Arthritis in Children

ARTHRITIS IN CHILDREN

 A booklet for parents and their children with juvenile idiopathic arthritis (JIA)

Introduction. 

I. Arthritis in Children, what is it? 

Juvenile Idiopathic Arthritis. What is it and why?

II. Juvenile Idiopathic Arthritis (JIA): 7 Different Types: 

(a) Systemic Arthritis

(b) Persistent Oligoarthritis (Oligoarthritis initially)

(c) Extended Oligoarthritis (Oligoarthritis initially)

(d) Polyarthritis, Rheumatoid Factor Negative

(e) Polyarthritis, Rheumatoid Factor Positive

(f) Enthesitis Related Arthritis

(g) Psoriatic Arthritis.

III. JIA: Systemic Arthritis 

IV. JIA: Oligoarthritis becoming Persistent Oligoarthritis or Extended Oligoathritis 

V. JIA: Polyarthritis, Rheumatoid Factor Negative 

VI. JIA: Polyarthritis, Rheumatoid Factor Positive  

VII. JIA: Enthesitis Related Arthritis (formerly Juvenile Ankylosing Spondylitis)

VIII. JIA: Psoriatic Arthritis 

IX. Eye Involvement 

X. Medications  

XI. Physical Therapy  

XII. Joint Injections  

XIII. Laboratory Tests and Investigations 

XIV. Questions Parents Frequently Ask 

ABBREVIATIONS

ANA – Anti-nuclear antibody

ANF – Anti-nuclear factor

CRP – C reactive protein

DMARD – Disease modifying anti-rheumatic drug

ERA – Enthesitis related arthritis

ESR – Erythrocyte sedimentation rate

FBC – Full blood count

LAARD – Long acting anti-rheumatic drug

JA – Juvenile arthritis

JIA – Juvenile idiopathic arthritis

JCA – Juvenile chronic arthritis

JRA – Juvenile rheumatoid arthritis

MRI – Magnetic Resonance Imaging

RF – Rheumatoid factor

SAARD – Slow acting anti-rheumatic drug

Introduction

When parents first learn their child has arthritis, it is a difficult time particularly when their child is very
young. The word “arthritis” sounds terrible, much worse than it actually is. Parents invariably imagine
old people seriously deformed with arthritis. The reality of arthritis in children is totally different and much
less gloomy. This booklet attempts to put the matter in perspective and to provide useful information for
parents, children, adolescents, other family members and those who are part of the support network.

Arthritis in children and adolescents is quite different from arthritis in elderly people. The majority of
children with arthritis tend to do well and many recover completely. It is the minority of children who will
be unlucky and have the disease continuing into adult life.

The more parents know and understand about arthritis in children, the easier it is for them to cope and
the better the outcome will be for their child. It is also very important for children and adolescents to
understand as much as possible about their own condition.

The most important thing all parents want to know is “What is going to happen to my child?” The next
question is “Why has this happened?” Though many questions about childhood arthritis remain
unanswered, it is hoped that this booklet will give useful and comforting information about the condition,
whilst being realistic and practical.

It has been a deliberate decision not to include too much detailed drug information, since drug therapies
are ever changing and information must be regularly up-dated. New medications are becoming available
continually and specific detailed information should be provided for each patient appropriate to the drug
regime planned. However, an attempt has been made to explain the broad groups of medications
available for children with arthritis with some specific information to facilitate an understanding of the
principles of treatment and the strategies involved.

SUMMARY OF NOMENCLATURE OF CHILDHOOD ARTHRITIS

JUVENILE IDIOPATHIC ARTHRITIS: arthritis present for at least 6 weeks commencing in a
child before the 16th birthday, with other known diagnoses excluded.

Juvenile Idiopathic Arthritis (JIA): 7 Different Types:

Systemic Arthritis

Persistent Oligoarthritis (Oligoarthritis initially)

Extended Oligoarthritis (Oligoarthritis initially)

Polyarthritis, Rheumatoid Factor Negative

Polyarthritis, Rheumatoid Factor Positive

Enthesitis Related Arthritis

Psoriatic Arthritis.

CHAPTER I

Arthritis in Children: What is it?

Juvenile Idiopathic Arthritis: What is it and why?

WHAT CONDITION DOES MY CHILD HAVE?

“Juvenile Idiopathic Arthritis” also at times referred to as “Juvenile Arthritis”

This was previously called Juvenile Rheumatoid Arthritis, but this term is used less frequently now
as it tends to give parents the wrong perception of a much more serious condition.

There are 7 different types of juvenile idiopathic arthritis, divided up by a number of things: the total
number of joints involved, some blood test results and special clinical features such as certain skin
rashes etc:

(h) Systemic Arthritis

(i) Persistent Oligoarthritis (Oligoarthritis initially)

(j) Extended Oligoarthritis (Oligoarthritis initially)

(k) Polyarthritis, Rheumatoid Factor Negative

(l) Polyarthritis, Rheumatoid Factor Positive

(m) Enthesitis Related Arthritis

(n) Psoriatic Arthritis.

Dividing the condition into subgroups or types helps to know the likely outlook, and how best to
prevent long term problems with regard to treatments.

In medical terms, “arthritis” means inflammation of a joint, which leads to heat, pain, redness and
swelling. The swelling is mostly from increased fluid in the joint (synovial fluid). There may also be
thickening of the joint capsule. In the normal joint there is only a small amount of synovial fluid and not
enough to be able to feel it with fingers when the joint is examined.

There are many different reasons why a joint may become inflamed. Hence there are many different
kinds of arthritis: for instance, if a joint is inflamed because of bacterial infection it is called “septic
arthritis” or “bacterial arthritis”. If it is due to viral infection, it is “viral arthritis”. If it is the form of arthritis
due to “wear and tear” commonly seen in the elderly, it is called “osteoarthritis” or “degenerative
arthritis.”

This booklet is about another form of arthritis found only in children. Its official name is juvenile
idiopathic arthritis (JIA), but the shortened term “juvenile arthritis” (JA) is also used. In these chapters,
JIA and JA are used interchangeably. The condition was previously called “juvenile rheumatoid arthritis”
(JRA), or “juvenile chronic arthritis” (JCA), but these terms are now being phased out as they tend togive parents the wrong impression about the condition. JIA is a better term. “Idiopathic” means it is a primary disease developing independently and we do not yet know the cause.

Definition: Juvenile Idiopathic Arthritis is arthritis present for at least 6 weeks commencing in a
child before the 16th birthday, with other known diagnoses excluded. There are no specific tests for
this form of arthritis and the diagnosis is made on a combination of clinical features and history. Blood
testing is used to exclude other conditions and to help define which form of JIA is present. (Refer to
Chapter XIII.)

We do not yet understand why JIA occurs. A healthy person has a complicated immune system to
protect from foreign invaders such as germs, splinters etc and to recognize what is “self” and what is
not. One of the functions of this part of the immune system (the autoimmune system) is to monitor and
deal with the presence of foreign bodies. This is accomplished by a delicate system of chemical signals
(cytokines) from one cell to another, with many cells and signals involved. Inflammation is one of the
early steps in this process.

When JIA occurs, it seems the autoimmune part of the immune system has made an error and an
incorrect signal (via chemical signals from cells in the blood) has been given and inflammation
commences in certain joints for no known reason. This is juvenile idiopathic arthritis, and inflammatory
condition of the body.

The trigger for the process is unknown, but it is most likely to be an infective agent of some kind. It is
thought that the infective agent will eventually be identified, and will not be a single agent, but several
different ones any one of which is able to trigger the condition. The joints however, are not infected. If
infection has occurred elsewhere it is likely to have passed by the time arthritis becomes apparent in the
child.

A genetic susceptibility for arthritis may pre-exist in a particular child for JIA to occur. However, there is
one main form of JIA, (enthesitis related arthritis previously called juvenile ankylosing spondylitis), where
genetic susceptibility is well defined and is regularly passed down. The gene marker called “B27” is
present in most children with this form of JIA and can be found with a simple blood test. The
susceptibility for psoriatic arthritis may also be passed on. However, in general, most children with JIA
will not pass it to future generations, nor do brothers and sisters have an increased risk of developing
JIA.

JIA is divided into 7 different types or subgroups depending on a number of factors such as how many
joints are involved, whether there have been high fevers at onset, family history of psoriasis and other
conditions, and the results of blood tests for rheumatoid factor and B27 antigen. Each of the subgroups
has a different set of potential problems. Thus it is useful to know which subgroup each child’s condition
fits, so that the best prediction of outcome can be made, and most appropriate treatments planned. As
new medications are developed the outlook improves.

Sometimes it is not possible to classify JIA into the correct subgroup until at least 1 year of disease has
passed. Sometimes the disease does not exactly fit into any of the defined subgroups of JIA.

The first thing parents want to know is what will happen to their child. Classifying JIA into subgroups
allows this question to be better answered. Even so, there is no sure way of knowing the exact outcome
for each child.

Viral arthritis or transient arthritis is more common in children than JIA and usually lasts about 2 weeks,
but sometimes longer. For a definite diagnosis of JIA to be made, the arthritis must have been present
for at least 6 weeks and thus viral arthritis has been excluded.

How often does JIA occur in children? It depends what one reads, but a West Australian study
performed on children at school showed that one in every 250 children had the condition. For some of
these children, the condition was mild and at the time of the study the parents had not actually been aware that the child had had a swollen joint for at least 6 weeks. However, for others the condition is much more obvious, and parents and children are definitely aware that something is amiss. Over the last few years, as awareness of the condition has increased, it has become accepted that JIA is not a rare disease.

It is important to note that many children with arthritis, particularly small ones, never complain of pain in
the joints. This is surprisingly common. It will then take longer for parents to notice there is something
abnormal about the child’s joints because the child is not complaining and there does not seem to be
anything wrong. A delay in diagnosis frequently occurs in this situation, and parents should be
reassured that this is not unusual, and does not mean that the child has been neglected.

CHAPTER II

Juvenile Idiopathic Arthritis: 7 Different Types

There are 7 different types of Juvenile Idiopathic Arthritis, each with different characteristics and with a
different outlook. Classification into subgroups makes it easier to predict outcome and plan appropriate
treatments.

The classification system is not particularly precise and is constantly being up-dated as new information
becomes available. An international committee regularly reviews the classification to ensure it is the best
possible classification available in light of current knowledge. Despite its shortcomings, it is a useful
guide for clinicians to plan management, and allows more precise research internationally. The 7
subgroups include the following:

(a) Systemic Arthritis

(b) Persistent Oligoarthritis (Oligoarthritis initially)

(c) Extended Oligoarthritis (Oligoarthritis initially)

(d) Polyarthritis, Rheumatoid Factor Negative

(e) Polyarthritis, Rheumatoid Factor Positive

(f) Enthesitis Related Arthritis

(g) Psoriatic Arthritis.

The classification depends on family history, clinical history for the particular child, which joints and how
many are involved and certain blood tests, which are discussed in detail in chapter XIII. At the first
medical consultation for the child with arthritis, it may not be possible to know in which subgroup the
child’s condition belongs until blood test results are available.

In effect, there are 2 names that are correct terms to describe each child’s condition: the overall name
“juvenile idiopathic arthritis” and also the subgroup e.g. “persistent oligoarthritis”. This can be confusing
but most parents when referring to their child’s condition will use the terms “juvenile idiopathic arthritis”,
“JIA” or use the shortened form “juvenile arthritis”.

Each subgroup of JIA is discussed individually in the following chapters. Only one such chapter is likely
to be directly relevant to each child.

CHAPTER III

Systemic Arthritis

Definition of systemic arthritis: Arthritis in one or more joints with, or preceded by, daily fever of
at least 2 weeks duration that is documented to be quotidian for at least 3 days, and accompanied
by one or more of the following: evanescent rash, generalised lymph node enlargement,
hepatomegaly or splenomegaly or serositis.

(Note: “quotidian” means recurrent and spiking; “evanescent” means changeable.)

This form of JIA is quite distinct in the beginning because of the typical high spiking fevers and very sick
children. Years ago it was called “Still’s disease”, after the English doctor who first described it in 1896.
The term “Still’s disease” is seldom used in current medical literature.

Because the children with systemic arthritis are sick in the beginning, they are usually taken to the doctor
promptly. Systemic arthritis can occur in a child of any age, including infants but most commonly it
occurs between the ages of 1 and 5 years. Boys and girls are affected in equal numbers.

There are 2 basic components to systemic arthritis, the systemic component (i.e. the child is sick with
fevers, rashes etc and is miserable), and the arthritis component, where the joints become swollen and
painful. The systemic component happens first. The joint component (i.e. pain and swelling of the joints)
may be present in the beginning or it may begin a few weeks later.

The fever tends to spike daily at a regular time. Sometimes the spikes occur twice per day, and
occasionally every second day. When the temperature is high, the child is sick and miserable. A few
hours later, when the temperature is down, the child may seem normal. The change in the child over a
few hours can be dramatic.

Usually there is a faint rash, which becomes more noticeable with high fevers. The site of the rash
changes constantly. It is soft pink, slightly raised, rounded or oval, varying in size from that of a currant,
to that of a ten-cent piece and is usually in clumps. There may be a darker border around some spots.
The rash is usually not itchy.

The child may have swollen lymph glands and enlargement of the liver and spleen.

One of the most serious features is pericarditis, being inflammation of the sac around the heart, causing
fluid accumulation in the sac. This causes chest pain and breathlessness and is usually accompanied by
a racing pulse. Pericarditis occurs only rarely.

The systemic illness may last for years if untreated, but on average it lasts for 2 or 3 months. It may recur
on a regular basis, eg once every few months. In most children it settles with treatment moderately
easily.

In the beginning, joints may seem normal though there may have been complaints of pain by the child.
The swelling of the joints usually starts 2 or 3 weeks after the start of the fevers, but can occur anytime
from the first day of the illness to several months later. There may be one or two joints involved or many
joints including finger and toe joints, back, neck, knees, hips, ankles, elbows, wrists etc.

Investigations

Blood tests usually show a high ESR, high white cell counts and low haemoglobin level. Rheumatoid
factor (RF) is not present in the blood, and likewise anti-nuclear antibody (ANA) is usually absent. (Refer
to Chapter XIII.)

Management
The management of the systemic part of the disease (fevers, rash etc) is different from the management
of the joint problems and it is useful to consider the treatment for each of these components separately

Management of systemic illness: The fevers are debilitating. Anti-inflammatory medication will often be
tried first to control the fevers, and need to be given at very regular intervals. For many children these
prove ineffective, and corticosteroids will be required to control the fevers and general symptoms. Many
rheumatologists will not attempt to use anti-inflammatory medication in this situation and will start with
corticosteroids as soon as the diagnosis is established. Corticosteroids can be given in a number of
different ways to control systemic manifestations i.e. fevers, rashes, general unwellness etc. Using oral
steroids was previously the most common method, given daily in a single or divided dose or given
second daily. There is now an increasing tendency to deliver corticosteroids by intravenous pulsing in
large doses usually 3 doses given over 3 to 5 days. This method is powerful and seems to cause less
side effects than daily oral doses. The aim is to get the maximum effect from the steroids with minimum
side effects. The amount of corticosteroid required by each individual, to bring the systemic
manifestations under control is variable. Some children will also require steroid-sparing medications in
addition to steroids. These include medications such as cyclosporine.

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.

Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.
  • Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overview of management for systemic arthritis: Most children will have corticosteroids in some form
in the early stages of the disease when systemic features are most prominent. As time goes by and the
joint problems become more evident, most children will have anti-inflammatory medication and many will
require second line agents to bring the condition under control.

Long-Term Outcome

The outcome is variable. Some children will have only a short illness and within a few months be back to
normal. Some children will have regular recurrences of systemic features (fevers, rash etc) over a
number of years. Some children will have only on-going joint problems. In a long-term study of adults
who had systemic arthritis as children, it was found that 65% had significant functional problems in
adulthood (Packham & Hall 2002). The long-term figures would now be much improved since there is
access to better medications, and fewer adults will have problems from having had the disease in
childhood. The exact outlook for each child is difficult to predict in the early phases of the condition.

 

CHAPTER IV

Oligoarthritis which becomes Persistent Oligoarthritis or
Extended Oligoarthritis

Definition of oligoarthritis: Arthritis affecting 1- 4 joints during the first 6 months of the disease.
Two subcategories are recognised: 1. Persistent oligoarthritis: affecting no more than 4 joints
throughout the disease course. 2. Extended oligoarthritis: affecting a cumulative total of 5 joints
or more after the first 6 months of disease.

(If the following are present the diagnosis of oligoarthritis is excluded and another diagnosis is
considered: psoriasis, family history of psoriasis, family history of HLA B27 related disease,
presence of rheumatoid factor, presence of systemic arthritis as described earlier.)

OLIGOARTHRITIS

In the first 6 months of the condition, a diagnosis of oligoarthritis is made when there are 4 or fewer joints
involved and if the conditions listed above have been excluded. Some children will never have more than
4 joints involved and after 6 months or so the disease is then called persistent oligoarthritis to distinguish
it from the condition in other children that starts off in 4 or fewer joints, but after 6 months or longer, 5 or
more joints become involved. This latter form, called extended oligoarthritis, tends to be more severe
than persistent oligoarthritis, and the treatment regimes therefore tend to be more aggressive. The
diagnosis of oligoarthritis is used till it becomes clear whether the child has persistent or extended
oligoarthritis.

PERSISTENT OLIGOARTHRITIS

Definition of persistent oligoarthritis: Arthritis affecting no more than 4 joints throughout the
disease course.

This form of JIA tends to be the most mild of the forms. It can start in very young children of less than
one year. Typically, the children are in good general health. They seldom complain of pain and often the
first thing noticed by the parents is a limp, which has come on gradually if the involved joint is in the
lower limb. Sometimes it is others outside of the family who first notice a limp. It is not understood why
children often have little pain, but it is partly because they promptly stop doing the movement that hurts
them and that is why they limp if it is a joint of the lower limb that is involved. The commonest joints
involved in this form of arthritis, are one or both knees. Other joints frequently involved include ankles,
wrists, elbows, fingers and toes, but any combination can occur.

Eye Disease

Children with this form of JIA may develop chronic uveitis or iritis, which means inflammation of the eyes.
(Refer to Chapter IX.) Usually there are no symptoms though a child may find bright sunlight causes
discomfort. The eyes are usually not red though occasionally they are. Even though the eyes are
asymptomatic, considerable damage can occur in the eyes unless treatment is given. Hence, the eyes
must be checked regularly by an ophthalmologist who uses a slit lamp being a special light to examine
the child’s eyes looking for inflammatory cells in the fluid within the eye. Treatment is usually
corticosteroid eye drops. The majority of cases respond well. When the inflammation proves difficult to
control with corticosteroid drops, other medications may need to be given. Few children in Australia have
long-term visual handicap from the problem.

Chronic uveitis occurs in approximately 20 % of Australian children with oligoarthritis. It is most likely to
occur in children less than 7 years of age at onset of arthritis, and in those who have tested positive for
ANA in the blood. Uveitis occurs more often with oligoarthritis than with any other subgroup of JIA.

Investigations
Blood testing may show a normal full blood count and the ESR is either normal, or moderately raised.
The ANA is positive in approximately 50%. Early in the disease, x-rays will be normal. Later, there may
be changes visible on x-ray. (Refer to Chapter XIII.)

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.

Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.
  • Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overview of management for persistent oligoarthritis: Joint injections are used commonly and are
very effective. Most children will have anti-inflammatory medication and a few only will require second
line agents. Physiotherapy is an important component of the treatment regime as are the regular eye
reviews.

Long-Term Outcome

In the majority of cases, the arthritis settles completely after a number of years, with no further
recurrences and the children return to normal health. The aim of treatment is to prevent joint damage
and deformities, so that when the condition does go away, the joints are normal. The commonest
abnormality to occur is where one knee has arthritis and one knee remains normal. The leg with the
arthritis of the knee may become longer, due to increased blood supply around the affected knee, which
stimulates the bone growth plate near the knee, and causes excessive growth. Usually, the difference
between the lengths of the two legs is minor but can become serious if overgrowth continues unchecked
for extended periods of time. Joint injection into the inflamed knee is particularly effective and is used to
prevent the leg from overgrowth. If the knee inflammation is kept under control, over a period of months
and years, the legs tend to equalize in length in the majority of children, unless a large difference in
length had developed. The long-term outlook is very good for children with this form of the disease and
most will have completely recovered by the time they become adults.

EXTENDED OLIGOARTHRITIS

Definition of extended oligoarthritis: Arthritis affecting fewer than 5 joints in the first 6 months of
the disease, and then a cumulative total of 5 joints or more after the first 6 months of disease.

In the beginning of the disease, the children have oligoarthritis. It is not possible to predict in the first few
months whether the number of involved joints is going to increase. However, usually the number of joints
will have increased beyond 4 by the end of the first year if the diagnosis has become extended
oligoarthritis. This condition is usually more severe than persistent oligoarthritis. Approximately one third
of the children who start with oligoarthritis develop into extended oligoarthritis. This condition may need
more aggressive treatment, as the arthritis may become severe.

Eye disease

Uveitis can also occur particularly if the child is positive for ANA on blood testing. Regular eye checks
are necessary. (Refer to Chapter IX.)

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.
Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.

Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overview of management for extended oligoarthritis: Many children will be given second line
medications early in the course of the disease, since it is known that this form of JIA can be quite
aggressive leading to joint damage. The treatment regime needs to be fairly aggressive also. As with
most forms of JIA, there is an increasing place for biological agents later if other treatments have not
been effective. Joint injections are often used also.

Long-Term Outcome

Not all children with this type will have serious joint disease, but many do. Some will recover completely
and the disease will go away. Some children will have ongoing joint disease, and some will develop
severe deformities in certain joints. In a long-term study of adults who developed extended oligoarthritis
as children, it was found that 36% had significant functional limitations as adults (Packham & Hall 2002).
However, with newer and more powerful medications, the disease is better controlled now and fewer
adults will have problems from having had this condition as children.

CHAPTER V

Polyarthritis Rheumatoid Factor Negative

Definition: Arthritis affecting 5 or more joints during the first 6 months of disease; tests for RF
are negative.

(If the following are present the diagnosis of polyarthritis (rheumatoid factor negative) is
excluded and another diagnosis is considered: psoriasis, family history of psoriasis, family
history of HLA B27 related disease, presence of rheumatoid factor, presence of systemic arthritis
as described earlier.)

In polyarthritis (rheumatoid factor negative) there are more than 5 joints involved from the beginning. It
can start at any age and girls develop it more often than boys. The children are sometimes mildly unwell
in the early stages, but mostly are in good general health. The first thing noticed is swelling of several
joints, which may or may not be painful. The pattern of joint involvement is variable. Sometimes the
disease is symmetrical i.e. the same joints are involved on each side of the body. There may be many
finger and toe joints involved, and also large joints such as knees, ankles etc. If the neck is involved it will
be stiff and painful. The children may be stiff and sore particularly first thing in the morning. They may
sometimes have a low-grade fever. Like most forms of childhood arthritis, it can become temporarily
worse with a passing infection such as a cold or ‘flu.

Investigations

The FBC and ESR may be normal. The ANA is usually negative and the RF is negative. (Refer to
Chapter XIII.)

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.

Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.

Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overveiw of management for polyarthritis rheumatoid factor negative: Most children will have anti-
inflammatory medications and a moderate number will be given second line medications. Many children
will have joint injections for selected joints. Uveitis occurs occasionally in this subgroup in Australian
children. The most common practice is for annual eye reviews by an ophthalmologist. If the child is very
young, the eyes may need to be checked more frequently.

Long-Term Outcome

Fortunately, the majority of children eventually recover from this type of arthritis, with the duration being
variable (months to years). Some will recover completely with no joint damage. Some may have residual
limitation of movement in the affected joints of varying severity. Whilst this condition is a nuisance, on the
whole the children are not sick, their daily lives are not necessarily hampered, and they should not miss
much school because of the condition. A long-term study of adults who developed polyarthritis
(rheumatoid factor negative) in childhood (Packham & Hall 2002) showed that 41% had functional
problems as a result of the arthritis. However, the current figures would be much improved on this,
because of newer medications available and more aggressive regimes undertaken. Hard work on a
regular basis, (i.e. physiotherapy) improves the end result significantly. Even in the more severe cases,
the child can expect to have a good outlook and hopefully have minimal joint damage.

CHAPTER VI

Polyarthritis Rheumatoid Factor Positive

Definition: Arthritis affecting 5 or more joints during the first 6 months of disease associated with
positive rheumatoid factor tests on 2 occasions at least 3 months apart in the first 6 months of
the disease.

(If the following are present the diagnosis of polyarthritis (rheumatoid factor positive) is excluded
and another diagnosis is considered: psoriasis, family history of psoriasis, family history of HLA
B27 related disease, presence of systemic arthritis as described earlier.)

This is the most rare and most severe form of juvenile idiopathic arthritis, occurring in 5-10% of children
with JIA, most commonly in teenage girls. However, boys and young children can also develop it.

As with other types of JIA, in the first days of the condition, the child may be mildly unwell. Pain and
swelling of the joints are noticed, often in the small joints of the hands and toes and elsewhere in the
body, eg. wrists, elbows, knees and ankles. The arthritis is often symmetrical. X-rays taken will be
normal at first, but it is possible that within 12 months some damage may be evident if treatment has not
been effective. The aim of treatment is to prevent damage. Anticipating possible damage is important,
since appropriate treatment measures can be put in place early to prevent or minimise damage.

When the child or adolescent develops an intercurrent illness, the joints might “flare” temporarily. The
term “flare” is used to describe a time when the joints have become dramatically worse over a short
period of time. If the flare is due to a viral illness, such as the flu’, there is a chance that the joints will
improve quickly again, after the viral illness has passed.

Investigations

Rheumatoid factor is always present, by definition. The FBC is normal or near normal. The ESR may be
moderately elevated or normal. The ANA may be positive or negative. (Refer to Chapter XIII.)

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.

Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.

Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overview of management for polyarthritis rheumatoid factor positive: Anti-inflammatory medication
will be required for most children. In addition, second line medications tend to be given early and the
treatment is generally fairly aggressive. Some children will be offered biological agents, later if the
second line medications have not been effective. Most children with this form of JIA will have their eyes
checked annually. Few develop uveitis.

Long-Term Outcome

Young people who suffer from this condition rarely have a short illness and it is known there is a
significant risk of joint damage without prompt and effective treatment. A long-term study of adults with
childhood onset of this form of JIA showed that 40% had functional problems with their joints. However,
with the early use of second line agents and with the availability of newer medications, the outlook is
greatly improved. As with all forms of the disease, hard work with physiotherapy allows the joint function
to be better maintained.

When career choices are being considered, it is wise to plan a career with minimal manual labour
involved, in case there is joint damage that makes physical labour more difficult. Education is very
important, as it allows wider employment options. This is the same for all young people.

There is a possibility that a condition called “amyloidosis” could develop later in the disease course. It
causes deposition of a substance, “amyloid”, in the kidneys and elsewhere and is potentially serious. In
Australia it occurs very rarely if at all. The reason for this is not understood.

CHAPTER VII

Enthesitis Related Arthritis (ERA)

Previously known as Juvenile Ankylosing Spondylitis

Definition: Arthritis and enthesitis,

or

Arthritis or enthesitis with at least 2 of the following: (a) sacroiliac joint tenderness and/or
inflammatory lumbosacral pain (b) presence of HLA B27 antigen (c) onset of arthritis in a male
over 6 years of age, (d) family history of ankylosing spondylitis, enthesitis related arthritis,
sacroiliitis with inflammatory bowel disease, Reiter’s syndrome or acute anterior uveitis.

(If the following are present the diagnosis of enthesitis related arthritis is excluded and another
diagnosis is considered: psoriasis, family history of psoriasis, presence of rheumatoid factor,
presence of systemic arthritis as described earlier.)

(Note: “enthesitis” means inflammation of the site where tendons attach on to bone.)

This form of JIA, generally starts with swelling of 2 or 3 joints, usually of the lower limbs, such as the
knees, ankles or hips and typically occurs in older boys. Sometimes it starts with painful heels or soles of
the feet or there may be a swollen toe or finger. Occasionally, it commences with neck stiffness and pain,
back problems or chest pain, though significant neck and back involvement is usually a later
development. Enthesitis causes pain and swelling at the sites of attachment of tendons to bones. It most
commonly occurs at the back of the heels where the Achilles tendon attaches to the heel bone. It may
also occur under the foot where the plantar fascia attaches or at the top of the pelvic bone (iliac crest)
where muscles attach.

The condition may “come and go” and the child may be completely free of any problems for long periods
of time. During these intervals the child is likely to be off all medication.

It was said that boys were affected much more frequently than girls, but this is now being questioned.
Likewise, the usual age of onset was said to be after 6 or 8 years, but it is known that children as young
as 2 years may show the first signs of it.

Inflammation of the eyes occurs. It is most commonly acute inflammation causing pain and redness of
one or both eyes (acute uveitis). However, occasionally, the silent type of eye inflammation (chronic
uveitis) may occur and this is difficult to diagnose as there are few symptoms. Regular eye reviews may
therefore be advisable on an annual basis. (Refer to Chapter IX.)

Investigations

In more than 90% of cases, the child will show the HLA B27 antigen on blood testing. Occasionally,
children can have this disease when this test is negative, but it is difficult to make a firm diagnosis if so.
All other investigations may be normal, though the inflammatory indices (ESR and CRP) are often
elevated. (Refer to Chapter XIII.)

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.

Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.

Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overview of management for enthesitis related arthritis: Physiotherapy is very important in this form
of JIA especially if there is back and neck involvement. It is known that fusion of the back and neck can
occur in a few individuals, despite treatment with medications. This however, seldom happens in
childhood or adolescence. It should be understood that if the back and neck do have fusion between
some vertebrae, so long as straight posture has been maintained, little inconvenience or problem will
result. Physiotherapy and special back exercises and the use of a firm mattress at night helps maintain
straightness of the spine. In days past, fusion might occur with the spine bent, and this would be very
awkward. This seldom occurs now and need not happen. It should be stressed that fusion of parts of the
spine is rare in childhood in any case. Most children and adolescents with the condition will be on anti-
inflammatory medication intermittently or continuously. Second line agents will be added if the condition
is persisting. Biological agents have been found to be effective and are used for the most severe forms
of the disease with excellent results in most.

Long-Term Outcome

There is a wide range of possible outcomes for this form of JIA. In the mildest form, the child may be free
of symptoms of the condition most of the time, with a painful swollen joint now and then. In the most
severe form, many joints may be involved and there may be fusion between a few of the vertebrae of the
spine. If the posture is good, this makes little difference to the child or adolescent. All children and
adolescents with ERA should be alerted to the possibility of the occurrence of acute iritis, so that
appropriate medical attention is sought for a painful red eye.

CHAPTER VIII

PSORIATIC ARTHRITIS

Definition: Arthritis and psoriasis or arthritis and at least 2 of (a) dactylitis, (b) nail abnormalities
(pitting or onycholysis), (c) family history of psoriasis in a first degree relative.

(If the following are present the diagnosis of psoriatic arthritis is excluded and another diagnosis
is considered: arthritis in HLA B27 positive male after 6th birthday, family history of HLA B27
related disease, presence of rheumatoid factor, presence of systemic arthritis as described
earlier.)

(Note: “Dactylitis” means swelling of a whole digit, rather than swelling of joint only; “onycholysis” means
lifting of the fingernail.)

There is a wide range of severity of this condition. Some children will have only one joint with arthritis
that is easily controlled with intra-articular corticosteroid injection. Other children may have many joints
involved with potentially severe destructive arthritis. The severity of the psoriasis does not necessarily
reflect the severity of the arthritis. Dactylitis and nail changes occur frequently in psoriatic arthritis. Most
commonly the children are in good general health despite arthritis.

Some children with psoriatic arthritis will develop uveitis, which at times can be severe. All children with
this form of JIA should have regular eye review by an eye specialist. (Refer to Chapter IX.)

Investigations

The ESR and CRP may be elevated or may be normal. The gene HLA B27 is present in a proportion of
children with psoriatic arthritis. The ANA is usually negative, and RF is not present. (Refer to Chapter
XIII.)

Management of joint inflammation:

Drug therapy may include:

  • Anti-inflammatory medication aimed specifically at combating inflammation, which is swelling, heat, pain and loss of movement. There are many different anti-inflammatory medications and new ones are developed continually with the aim of lessening side effects and improving efficacy.
  • Second line medications given when the condition is more severe and requires more than symptomatic treatment. These medications attempt to “turn off” the disease process by affecting the immuno-regulatory system of the body. They are given only after careful consideration. Their use is monitored closely and regular blood tests are required. There are increasing numbers of such medications. They have significantly improved the outlook for children with JIA.
  • Biological agents are the most recent type of medications that have become available for treating JIA. These are complex, very expensive medications, and can only be used when other medications have failed to bring the condition under control.
  • Corticosteroid administered in various ways.

Refer to Chapter X for further information regarding medications.

Local therapy includes:

  • Joint injection using corticosteroids useful for certain individual joints.

Refer to Chapter XII for further information on joint injections.

Physiotherapy:

  • Physiotherapy is an essential part of treatment particularly where limitation of movement due to inflammation is likely to occur.
  • It aims at maintaining function of the joints i.e. the maximum range of movement around the joints with good strength of adjacent muscles.

Refer to Chapter XI for further information.

Overview of management for psoriatic arthritis: There is a very wide spectrum of disease severity,
and the treatment regime is fashioned accordingly. Many children will need little more than joint
injections, whilst others will need more comprehensive drug regimes with second line agents included.
Biological agents are also appropriately used for the most severe when other medications have not been
effective. Regular eye reviews are important as eye involvement can occasionally be severe.

Long-Term Outcome

This is very variable. The arthritis may be mild and restricted to one or two joints. It may be widespread
in many joints and be severe and potentially destructive. Many children with psoriatic arthritis will have a
similar disease course as for children with persistent oligoarthritis. A few children will have a disease
course that resembles enthesitis related arthritis (see in earlier chapters). With newer medications and
biologics, the outlook is greatly improved.

CHAPTER IX

Eye Involvement

With some forms of juvenile idiopathic arthritis, inflammation occurs not only in the joints, but also in the
eyes and is called “uveitis” or “iritis”. It occurs more frequently in children with oligoarthritis, particularly
where the child is young and is positive for ANA on blood testing.

The uveitis is mostly in the chronic form, which can be largely without symptoms, even though significant
damage to the eye may be occurring. For some children, there may be slight redness, blurred vision, and
a dislike of bright lights. Acute uveitis also occurs, particularly in the subgroup enthesitis related arthritis.
In this form the eye is acutely painful and red.

In some countries it has been reported that uveitis occurs in nearly 50% of children with oligoarthritis
mostly where ANA is present, and in girls more often than in boys. In Australia most centres report about
20% of children with oligoarthritis have the associated eye problem. In a study by Packham and Hall
(2002) the following percentage of children in each subgroup were found to have uveitis: Systemic:
3.8%; Persistent oligoarthritis: 46.7%; Extended oligoarthritis: 40%; Polyarthritis rheumatoid factor
negative: 24.4%; Polyarthritis rheumatoid factor positive: 2.7%; Enthesitis related arthritis: 28.1%;
Psoriatic arthritis: 20%. This study involved British children where the reported prevalence for uveitis is
significantly higher than that reported in Australia.

The frequency with which routine eye screening is undertaken is determined according to the type of JIA
present, the age of the child at onset of the condition, and whether the child is positive for ANA. The
American Academy of Pediatrics recommends the following for screening for uveitis:

Risk Level

Type of Juvenile Idiopathic Arthritis

Frequency of screening

High

Oligoarthritis, Polyarthritis <7 years at onset, ANA +ve

Every 3-4 months

Medium

Oligoarthritis, Polyarthritis <7 years at onset, ANA – ve

Every 6 months

Medium

Oligoarthritis, Polyarthritis >7 years at onset, ANA + or –

Every 6 months

Low

Systemic arthritis

Every 12 months

Though uveitis typically occurs at the beginning of the illness, it is known that it can occasionally occur in
a susceptible child, after JIA has gone into remission. Hence, annual eye checks should continue in
certain children after the arthritis has settled. Such children include those with oligoarthritis or
polyarthritis who were of young age at onset of JIA, particularly those who were ANA positive. How long
eye checks should continue is debatable since the chance of occurrence of uveitis later is low. It is the
policy at some centres to continue eye checks in the most susceptible until age of 12 or 13 years.

Management of uveitis consists of corticosteroid eye drops, plus medications if necessary. These include
anti-inflammatory medication, second line agents and sometimes biological agents in the most severe,
where other treatments have not been effective.

Untreated, there is a risk of severe damage and visual handicap. Blindness remains a theoretical risk but
in reality it does not occur at least in Australia, where severe uveitis is a rare occurrence and can now be
treated much more effectively than in the past.

CHAPTER X

Medications

This section is designed to give parents and older children a broad understanding of the different types
of medication and the reasons for their use in the management of JIA. It is not intended that the latest
information on the latest medications be provided here, since detailed drug information constantly
changes. Parents should attempt to obtain detailed and current information on all medications their child
is given.

The internet is a valuable source of information and parents should be encouraged to use it. However
parents must also be careful. Internet sources are not necessarily kept up to date and certain sources
may not be reliable. In addition there is a lot of misinformation on the net created by people promoting
their own biases. It is useful to discuss material taken from the net with the child’s doctor, but this can
also be time-consuming for the doctor who can not hope to read all the material that is made available.

Medications are used to:

  • Treat symptoms
  • Improve quality of life for the child
  • Terminate the disease process if possible
  • Limit any damage to joints or other parts of the body.

It is normal for parents to worry about their children being on regular medications. However, it is useful
for parents to realise that no parents want their children to be on unnecessary medications but neither do
parents want their children to have to suffer from arthritis.

Medications given correctly help children overcome disease and minimize symptoms that the children
otherwise suffer. It is a matter of working out what is best and safest for each individual child and getting
the balance right. It is sad to see children suffering unnecessarily where medications could help, but are
being withheld for the wrong reasons. If medications are safe and effective, children should be given the
medications they need.

There are 5 main categories of medications used in the treatment of JIA:

A. Anti-inflammatory medications

B. Second line medications, also called slow-acting anti-rheumatic drugs (SAARD’s), long-acting
anti-rheumatic drugs (LAARD’s), disease modifying anti-rheumatic drugs (DMARD’s) or remittive
drugs

C. Biological agents

D. Analgesics

E. Corticosteroids.

A. Anti-inflammatory medications

Inflammation is a term used to describe the body’s reaction to certain agents that can damage the body.
Inflammation consists of heat, pain, redness, swelling and loss of function. Anti-inflammatory
medications are designed to overcome these symptoms. The medications work by inhibiting the
production of prostaglandins which are substances involved in many body processes, one of which is
inflammation. Correctly used, anti-inflammatory medications are relatively safe. These medications do
not take away the disease process, but minimise the effect of symptoms of the disease on the joints, i.e.
minimise inflammation.

Most anti-inflammatory medications act not only on joints but elsewhere in the body, and sometimes on
the lining of the stomach. Gastric irritation can be a problem in about 20% of children. When this occurs,
some children will complain of abdominal pain, and some will lose their appetite. Taking milk or food with
the medication will help lessen the gastrointestinal side effects. Easy bruising is also a common side
effect because anti-inflammatory medications change the surface of platelets (one of the cells in the
blood involved in clotting). These medications will usually be discontinued 5 days prior to elective
surgery. However, in the case of emergency surgery for a child who has been taking regular anti-
inflammatory medication, the increased tendency for bleeding is mild enough that it does not usually
cause problems. However, if the situation should arise, parents should discuss this matter with the
doctors concerned.

The original anti-inflammatory medications are non-specific COX-1 and COX-2 inhibitors and include
medications such as diclofenac, ibuprofen, naproxen and piroxicam.

In recent years, a new type of anti-inflammatory medication has been developed that acts on the joints
but with less action on the stomach lining and on the surface of platelets. These medications are
generally not more powerful than the older ones, but have fewer side effects on the gastrointestinal
system. However, for some of the COX-2 inhibitors, an increase in cardiovascular side effects has been
shown and some medications in this class have been withdrawn from the market. In certain situations it
may be appropriate to use COX-2 inhibitors rather than the older type of non-specific COX-1 and COX-2
inhibitors, but only under careful medical supervision.

Aspirin is a form of anti-inflammatory medication. It has an additional possible side effect, Reye’s
Syndrome, a rare but serious condition of the liver. There is evidence to suggest that people taking
regular aspirin may be at increased risk of developing Reye’s syndrome, in the presence of a viral illness
(influenza or chicken pox). It is customary to advise that aspirin be ceased with these infections. Reye’s
syndrome may first become apparent by odd behaviour in a child who may also develop altered
consciousness as liver enzymes become elevated.

Anti-inflammatory medications are the most commonly consumed medication in the world.

Most children with arthritis will be prescribed anti-inflammatory medication as baseline medication whilst
there is joint swelling particularly if this is causing pain and limitation of movement and disruption to
quality of life of the child.

B. Second line medications

These medications are also called remittive medications, slow-acting anti-rheumatic drugs (SAARD’S),
long-acting anti-rheumatic drugs (LAARD’s), disease-modifying anti-rheumatic drugs (DMARD’S) etc.
They do not act directly on the symptoms or signs of the disease, which are the end result of the disease
process. Second line medications act by changing the immune system. The purpose of these
medications is to turn the disease process off, or at least down, at the point where the process begins,
which appears to be in the immune system.

These medications have some effect on the body’s capacity to fight infection, but at the doses given for
arthritis, few children have problems from increased infections. However, when a child is on second line
medications, it is important to discuss the matter of immunizations with the doctor, as live vaccines are
usually avoided.

Second line medications are powerful and have many side effects. They are used when JIA is not
improving, when quality of life is affected or when joint damage is occurring. Regular blood tests are
required and likewise urine tests for some of the medications. If the tests are not normal the drug may be
stopped temporarily or permanently.

Second line medications do not act immediately and may take weeks or months. None of them are
guaranteed to be effective for all patients. It is usually months before it is known whether the medications
are working. It is customary to increase the dose slowly to the required level. Since these medications
act slowly, and are acting at the source of the disease, rather than the end point (i.e. swollen and painful
joints), the daily dose is not changed according to symptoms. The following medications are included in
this group: methotrexate, hydroxychloroquine, cyclosporin, sulphasalazine, and leflunomide. New
medications are continually being developed.
The commonest drug of this type currently being used for juvenile arthritis is methotrexate. It has been
very useful and has changed the outlook of arthritis in children. It has been shown to be effective in
approximately 70% of children.

C. Biological agents

The newest advance in the treatment of severe JIA is the development of medications that specifically
target biochemical agents in the body (i.e. biological agents) that are known to be involved in the
disease process. The biologic agents are mostly given by injection. Mostly, these medications target
cytokines, which are biochemical substances used as signals from one cell to another whereby a chain
of events is initiated such as inflammation. Biological agents are very complex molecules that resemble
in part the structure of human molecules. They are very expensive to produce. They are used for severe
forms of JIA, uveitis and rheumatoid arthritis in adults, when other medications have been ineffective.

Most biological agents used currently, act against the cytokine called “TNF-.” and include etanercept,
infliximab and adalimumab. These prevent TNF-. signals getting to certain cells, and thereby prevent
inflammation and other actions of TNF-.. Some of the actions of TNF-. are desirable for the body, such
as helping in the fight against infections. Thus, when these medications are used, careful monitoring is
required, particularly in relation to possible infections. So far, infection has not been a big problem in
carefully controlled conditions. In some countries where latent TB occurs, reactivation of infection has
been known to occur with the use of these agents. It is known that patients on these medications can
develop autoantibodies. To date these have caused little problem in patients, but on-going surveillance is
required as they may predispose to autoimmune disease. As time passes other long-term side effects
may become apparent.

Other biologics target other cytokines such IL, IL6 or their receptors.

New anti-biological medications are being developed all the time. It is considered these are going to
become increasingly more important in treating rheumatological conditions in children and adults.

D. Analgesics

Analgesics (i.e. pain-killers) such as paracetamol, can be used at the same time as anti-inflammatory
medication since they act in a different manner to anti-inflammatory medications even though anti-
inflammatory medications also control pain. It is useful for the doctor to know how much analgesic
medication the patient needs. If a lot of analgesic is required, then the drug regime needs to be reviewed
and adjustments made to improve the pharmaceutical control of the disease.

E. Corticosteroids

Corticosteroids are the most potent anti-inflammatory agents used in rheumatological diseases. They are
also produced within the body and have multiple functions. These steroids are different from the steroids
taken illegally by some athletes to build up muscles. These are androgenic steroids or “male hormones”.

Corticosteroids are very powerful medications and have many side effects. They must be used with great
care, and always given in the least amount that is effective. The higher the dose and the longer they are
used, the more side effects occur. A small dose taken on a daily basis does not usually cause significant
side effects.

For systemic arthritis where there are recurring high temperatures in a sick child, corticosteroids will be
given either as oral medication or as pulses given intravenously. When the systemic features settle,
steroid doses will be reduced.

For some of the other forms of JIA, a small daily dose of oral steroids may be given to make the child
feel better, be less stiff and to have a more normal life. Given in this manner, the side effects are
minimal.

Corticosteroid eye drops may be necessary if uveitis is present with JIA. The steroid eye drops are
effective in many cases, but are not without side effects.

Corticosteroids are very useful given as an injection into joints. They are most effective and have minimal
side effects, given correctly. Their use in this manner has revolutionised the care of arthritis particularly
where only a few joints are involved.

Possible side effects of oral corticosteroids taken in reasonable doses over long periods of time, include
body and facial obesity, stretch marks, hypertension, acne, cataracts, elevated blood pressure, stunting
of growth, osteonecrosis (damage to parts of bones and joints) and other side effects. The list is
alarming, but most are reversible and many of them will not occur unless the dose is high and used for a
considerable time. In juvenile idiopathic arthritis, apart from systemic arthritis, it is rare for a child to have
to stay on corticosteroids on large doses for any length of time.
CHAPTER XI

Physical Therapy: Physiotherapy and Occupational Therapy

Inflamed joints tend to be painful when being moved through a range of movement. Hence children tend
to avoid moving such joints through the full range. Before too long, inflamed joints may become limited
and then it is not possible for the joint to move through the full range. Thus, it is important that if joints are
at risk of losing range, measures are taken to maintain the full movement and function of those joints.
The wider aim is to have each child participate fully in normal physical activities of childhood.
Physiotherapists and occupational therapists work closely together to achieve this.

Physical therapy falls into 3 main categories: exercising of the joints to maintain the range of movement,
exercising to strengthen muscles around joints, and splinting joints in certain positions for short periods
of time.

Exercising to maintain range of movement

Generally, physiotherapists recommend daily exercises for joints that are inflamed and are at risk of
losing range of movement. A few minutes per day, will make a difference.

Children tend to be reluctant to do repetitive exercises, as this is boring for them. Thus the best way to
exercise joints in children is to incorporate the exercise into daily activities, or fun activities or activities
shared with friends. This makes it easier for the child and for the parents.

Experienced physiotherapists and occupational therapists will devise a programme for each child
relevant to what joints need exercising. Not all inflamed joints require exercising. If a swollen joint
maintains full movement there may be no reason to undergo regular exercising of that joint.

Exercising to maintain muscle strength around inflamed joints

If muscles whose function is to move joints are not used regularly, they tend to lose size or “waste”. It
can happen quickly in children, over a period of 2 or 3 weeks. The wasting is generally temporary, and
can be overcome with regular exercising of the muscle, unless the condition has been present for a long
time in which case scaring and fibrosing may occur. However this rarely happens.

Splinting of joints

Some joints with arthritis may be splinted for some part of each day or during sleep. The splinting is to
encourage the joint to maintain a certain position in the range of movement, that may be under threat
because of arthritis e.g. knees are frequently splinted out straight, when a child goes to bed at night by
using a back slab. This is similar to half a cylinder that the leg rests in, and which is usually strapped on
with velcroe straps when the child is put to bed. The reason is that many knees when inflamed, tend to
be held in a flexed or bent position. With time, it becomes difficult for the child to fully straighten the knee
and the knee may become permanently bent. If at night time, the knee is maintained out straight for
some time regularly, then the range of movement is not lost.

Not all joints can be splinted and many joints do not need splinting. Knees and wrists are the joints most
commonly splinted for a part of the day or night. Generally a physiotherapist will assess each child to
determine which joints need splinting if any.

When a child has worn a splint at night, it is common for the joint to be stiff for a short period of time in
the morning immediately after the splint is taken off. The stiffness goes away after a short time as soon
as the child is up and about.

Hydrotherapy

Hydrotherapy is frequently used for children with arthritis. It consists of special exercises done in a
swimming pool of warmed water. Children generally enjoy this form of exercise especially when it is done
with other children. The warmth of the water also helps the joints to move better.

CHAPTER XII

JOINT INJECTIONS

Injection of corticosteroids into joints with inflammatory arthritis is a powerful and effective form of
treatment. It is particularly useful when there are only a few joints involved, such as knees, elbows, wrists
and ankles.

It should be noted that the particular form of steroid used in joints is not the same steroid used illegally by
athletes, these being male hormones useful for muscle building.

Corticosteroids are naturally made within the body and have multiple functions. By injecting steroids into
joints, the effect occurs at the joint with minimal side effects elsewhere in the body.

Joint injections are not appropriate for all children with arthritis particularly where there are many joints
involved or the child is unwell i.e. with fever etc. Certain joints are more amenable to injection than others
(knees, ankles etc).

Properly and carefully given, there are few side effects. There is a theoretical risk of infection, but with
the usual precautions, this is most unlikely. There can be a small area of calcification if some steroid
leaks out of the joint. This is rare, and does not usually cause any problems and disappears with time.
There can sometimes be a small mark on the skin where the needle has been inserted, particularly
around the ankles. This is also of no long-term importance.

When many joints are injected at the one time, children have been known to temporarily develop
increased appetite, and to develop mildly fuller cheeks (cushingoid). This may last for a month or two.

The joint injections are done under general anaesthetic, for young children and if there are several joints
to be injected or if it is hip joints being injected. Many injections will be done without a general
anaesthetic. Local anaesthetic cream or local anaesthetic injection may be used. A sedative may be
given as a medicine prior to the procedure to help the child relax. In some centres, “laughing gas” is also
used to make it easier for the child.

It is necessary to use an image intensifier, for certain joints. This is a form of x-ray, which ensures the
needle is in the right place for the injection. The image of the joint and the needle can be seen on the
screen as the needle is being inserted.

Children’s joints are injected less frequently than adult joints, because of concerns that multiple injections
may cause damage to the joint cartilage of children’s joints. However, there is little evidence for this and
many children have many joint injections without apparent damage. In contrast, we know that untreated
arthritis definitely leads to joint damage in many children. For best results, the timing of joint injections in
children is important. Some children may need to have an injection only once in a particular joint and
some may need to have a joint injected several times. Virtually all joints with inflammation will respond to
an injection of corticosteroids, but the duration of response is variable and difficult to predict. Its effect
may last from a few weeks to indefinitely.

Corticosteroid injections are a common form of treatment for juvenile arthritis, and they have helped
many children with arthritis.

CHAPTER XIII

INVESTIGATIONS AND LABORATORY TESTS

It is useful for parents to have some knowledge of the tests that may be required for their child. There are
no tests to prove “yes” or “no” for the diagnosis of JIA. The diagnosis is made on a number of things
including history, findings on clinical examination and by excluding other conditions.

Blood tests, whilst not confirming or excluding the diagnosis, are important to help to determine what
particular kind (i.e. subgroup) of JIA is present. This helps predict the future course of the child’s
condition and how serious it might be. Additionally, blood tests may be useful for monitoring disease
progress and for excluding other diagnoses.

The following are tests frequently done on children with arthritis or other autoimmune disease.

1. Full blood count (FBC), erythrocyte sedimentation rate (ESR) and C reactive protein (CRP)

These tests are non-specific and will show whether a child is anaemic and whether the red and white
blood cells are present in normal shape, size and numbers. A raised erythrocyte sedimentation rate
(ESR) may be an indication of significant inflammation or infection. A normal level however, does not
exclude them. The normal level for children is less than 15 mms per hour. For some diseases the ESR
can be 120 mms or higher. For children with JIA, ESR can be normal, particularly for oligoarthritis. In
untreated systemic arthritis it is always raised. In other forms of JIA, the ESR may or may not be raised.
The ESR can also be raised if a child has a bad cold or the “flu”. Thus checking the ESR is useful but
does not give information specific to arthritis.

Measurement of CRP has a similar purpose to measuring ESR. It is also non-specific for inflammation or
infection. It directly measures the level of a certain protein made in the liver, which can be increased
quickly if there is infection or inflammation in the body.

The meaning of both of these tests needs to be interpreted in the context of what else is happening to
the child.

2. Anti-nuclear factor (ANF) also called Anti-nuclear antibody (ANA)

This factor can be present in the blood of children who are apparently healthy. It therefore does not
necessarily mean disease is present. However, if it is present in high concentrations, it gives general
evidence that an autoimmune disease such as JIA is likely to be present. ANA is present in more than
90% of patients with systemic lupus erythematosus. The anti-nuclear factor is not responsible for causing
damage in JIA to the tissues (such as joints). Of all of the forms of JIA, ANA is most likely to be present
with oligoarthritis. It is useful to know whether a child with JIA is positive for ANA as it is associated with
an increased chance of the child developing uveitis.

3. Rheumatoid factor (RF)

Rheumatoid factor is a well-known blood test usually positive in adults with rheumatoid arthritis. Many
parents still think testing for RF is a useful test for arthritis in children. This is not correct, since RF is
present in less than 10% of children with JIA, usually in teenage girls with polyarthritis. Occasionally boys
or young children will also be positive for RF. Most children with JIA are checked routinely for RF in the
beginning, since it is important to know if it is present. Occasionally a child who has been negative for RF
will later convert to being positive. The reverse can also happen especially when a patient is treated with
the newer medications.

If the test for RF is positive, it is customary to repeat the test within 3 months for confirmation. A positive
RF indicates that the arthritis may be fairly severe.

4. HLA B27

The B27 antigen is the name of a product of one small gene of which everyone has many. Approximately
8% of Caucasian people have this gene always inherited from one of the parents. Each race has a
different percentage of the population having this gene eg it is said that less than 1% of Japanese carry
it. If a child has the gene, each brother and sister has a 50% chance of also inheriting the gene from the
same parents.

We know that being positive for B27, predisposes the carrier to developing certain diseases such as
ankylosing spondylitis, or the form of JIA called enthesitis related arthritis (the childhood form of
ankylosing spondylitis). It is not clear whether the actual gene producing B27, or a close neighbour of the
gene causes the susceptibility to these conditions.

Other diseases that are more common if B27 is present include psoriasis, iritis (inflammation of the iris of
the eye), and inflammatory bowel disease. This is different from “irritable bowel syndrome”. If a person
has B27, and does not have any signs or symptoms of these diseases, it does not necessarily mean they
will develop one of these conditions in the future.

To test for B27, a small blood sample is required.

The B27 antigen was originally discovered when organ transplants were first performed, and
compatibility between donor and recipient was being tested.

5. Diagnostic imaging (X-rays and Bone Scans etc)

X-rays of bones and joints are often done early in the course of arthritis or when the diagnosis is being
considered. They are useful to exclude other conditions and usually show no abnormalities in the early
phases of inflammatory arthritis. Later in the disease, changes on x-ray may be evident.

Bone scans give different information to normal x-rays. The commonest form of bone scans are
technetium bone scans which require an intravenous injection of a minute amount of radio-active
material, which is safe because the dose is small. An overheard camera scans the body as a child lies
on a bed for a short period immediately after the injection and again approximately 2 hours later. This
investigation outlines the blood supply to the skeleton and defines any areas of increased blood supply,
which occurs with infection, inflammation, injury or tumours of the bones. Thus bone scans are useful in
detecting or excluding a wide range of conditions.

Magnetic resonance imaging (MRI) is a sophisticated form of imaging that can show bones, and also
soft tissues such as muscles, tendons, nerves and blood vessels. It is very expensive and should only be
used when the more simple investigations have not been adequate. The patient lies on a narrow bed and
is surrounded by the magnetic machine and it is similar to lying in a small tunnel. Occasionally this can
be troublesome to people afraid of tight spaces. In addition, the machines are moderately noisy and
patients are usually given earphones to listen through during the procedure. The patient must lie still for
approximately half an hour. Children up to about 5 years may need a general anaesthetic for the
procedure, because they cannot lie still long enough. There is no radiation given in this procedure, and
the pictures show an amazing amount of detail. Sometimes an injection will also be given to aid in the
procedure when outlining certain structures.

CHAPTER XIV

QUESTIONS PARENTS FREQUENTLY ASK

1. Question: Why did my child get arthritis? Was it something I did, or did not do?

Answer: We do not yet know why a child gets arthritis. It is definitely not the fault of parents or child.
Some event triggers an abnormal reaction in the body’s immune system, which then causes the body to
set up inflammation in the body’s joints. It is considered that an infection is the most likely triggering
event. The part of the immune system that is constantly surveying the body for foreign invaders becomes
mistaken, and sets up a reaction in the joint. This is called “inflammation in the joint”, i.e. arthritis.
However, by the time JIA begins, it is considered that infection related to the arthritis, is no longer
present in the child.

2. Question: What is “autoimmune disease”?

Answer: There are a series of diseases where the basic problem is that the immune system is unable to
precisely prevent certain chain reactions within the body that are harmful to the body itself. Simply
stated, it is as if the body becomes confused about some small part of itself (such as a joint), and
considers this part needs to be challenged by inflammatory cells rather than to be left in peace. These
chain reactions in a normal situation are helpful and would help protect the body from harmful events
such as infection, invasion by a foreign body such as a splinter and other undesirable events. The body
is not really “attacking itself”, but it is generating an abnormal reaction that means some function of the
body is compromised and there will usually be symptoms. There are many different types of autoimmune
diseases. In JIA, the problem appears largely in the joints. In other types of autoimmune disease, the
problems appear in a variety of systems of the body.

3. Question: Can a change of diet cure my child?

Answer: No. The cause of arthritis is not a simple dietary problem, and therefore a change in diet will not
provide a cure in either children or adults. For juvenile idiopathic arthritis, no diet has been found to
cause or cure it. However, there is evidence to suggest that omega-3 fats (from marine food chains) are
beneficial to the body, and help reduce inflammation. These fats are also good for the heart and blood
vessels. They are present in fish in variable amounts. Tinned salmon is a good source of omega-3 fats.
Some authorities recommend 2 or more fish meals per week. This will not take away the arthritis, but it
may help lessen the inflammation to a minor degree. It is very important that a child has a balanced diet,
whatever else is in the diet. There are many “old wives tales” that blame tomatoes and other foods for
arthritis, but there is no evidence to support these claims.

Serum sickness is a form of allergy and is manifested by acute arthritis and an itchy rash in a sick child.
It can be caused by antibiotics and other medications and rarely by some foods. It usually lasts only 1 or
2 weeks and is quite different from juvenile idiopathic arthritis.

4. Question: Can “natural”, “alternative” or “complementary” medicines or treatments help my child?

Answer: This is a very difficult area of medicine about which much has been written. There are opposing
views and degrees of acceptance of the benefits of alternative medicines. It is beyond the scope of this
booklet to discuss at any length the various arguments for alternative medicines.

However, the overriding consideration is that parents must take great care when giving their child
medicines or treatments that have not been approved by mainstream medicine or proved scientifically.
They must know all that there is to know about the safety, and they must seek good scientific evidence
for likely benefit for the child.
Over the years, there have been thousands of alternative or natural medicines offered to parents of
children with juvenile idiopathic arthritis to cure their child. Often there is no scientific evidence that the
medicines are of benefit though this does not hold for all alternative medicines. Even so, it is a parent’s
right to try alternative or unproven medicines. Parents may get frustrated with conventional treatments
when they are not working well and will want to try whatever they can. However, no dangerous
compounds or treatments should ever be used and parents must be careful they know exactly what is in
the mixtures they are giving to the child. Parents must take full responsibility for what they are giving
since the substances may not have been subjected to rigorous testing nor government controls as for
conventional medicines which are subjected to testing for safety standards.

“Natural medicines” have come to mean substances extracted from nature such as trees and flowers.
However, there are some dangerous chemicals that can be extracted from plants such as poisons or
steroid-like chemicals. These may be called “natural”, because they come from plants, but they are
definitely not harmless. There is increasing amount of information available on alternative and natural
medicines and some professionals believe in the benefit of a restricted range of such medicines. The
internet provides much information for parents. Great care must always be taken that the source is
reliable, and the date is current.

Parents must be wary of any promises of a cure for arthritis. Science has not yet provided a cure.
Beware of expensive treatments that people will suggest. There are many dishonest people who
capitalise on vulnerable people such as parents who are worried about their child and most parents
would pay anything to have their child cured. If your child is on “alternative” or “natural” medicines, it is
helpful to keep the doctor informed.

5. Question: Will my child be physically handicapped or crippled?

Answer: No. The majority of children recover from JIA without significant damage to joints though some
children will have damage of varying degree. Improved medications have lessened the number of such
children and the majority will have a near normal life after arthritis. Joint replacement surgery is
sometimes necessary in teenage years or adulthood for damaged joints. Whilst the child has juvenile
arthritis, good treatments particularly good physiotherapy will ensure the child has the best chance of
maintaining normal functioning of the joints. It is useful for parents to presume that their child will recover
from juvenile idiopathic arthritis. The outlook varies between the 7 different kinds of JIA and some have a
better outlook than others. This is the main reason why it is useful to classify what type of JIA is present
in each particular child.

6. Question: Is my child likely to need joint replacement surgery?

Answer: Only the occasional child will need joint replacement surgery, usually in adult life, but
occasionally in teenage years for the more severe forms of JIA. This is most likely to happen where the
newer medicines have not been available for therapy and significant joint damage may have occurred.
The hips are the commonest joint replaced in young people. The surgery for this operation is continually
improving, and young people can have an excellent result. Hip replacement is very common in adults
and there are major advances in technique being made continually. Hip replacement in young people
has greatly improved the quality of life for many, since it allows them to have a normal social life and
normal mobility. There are much fewer joint replacements required in young people since better
medicines have become available for the treatment of arthritis.

7. Question: How can I discipline my child, now that my child has arthritis?

Answer: The child must be disciplined in exactly the same way as any other child. Arthritis should not
make any difference. He or she must be given limits and if those limits are exceeded, then the child must
pay the consequences as other children do. The damage done to a child, if he or she is not disciplined,
is far greater than any damage done by arthritis. Being firm with a child is not being unkind.

8. Question: Does juvenile idiopathic arthritis pass from one child to the next generation?

Answer: Generally no. Juvenile idiopathic arthritis is not passed on to subsequent generations except
for one form where the B27 is present. It is only the susceptibility that is passed on, not arthritis. Most
people who have the B27 antigen do not have arthritis but have an increased chance of developing
either juvenile idiopathic arthritis or ankylosing spondylitis in adulthood. Even so, the chance is not
particularly high. About 8% of the Australian population carry the B27 antigen and the majority of these
are healthy normal people.

9. Question: What about sport? Can my child play sports like other children?

Answer: Yes, more or less. It is very important for all children to play sport and participate in activities
with other children. Some common sense will be required to choose activities that are appropriate for the
child’s physical abilities. This is usually not difficult. Physiotherapists and occupational therapists will give
advice. The aim is to encourage and enable a child with arthritis to be the same as his peers, and to lead
a normal life. However, it would not be sensible for a child with arthritis in the knees or ankles to do
marathon running events, etc. It is helpful to remember that if a joint is slightly painful, then it is unlikely
that a particular activity is causing damage. If a joint becomes very painful because of a certain activity,
then this activity should be lessened.

10. Question: Many parents say, “We are not the kind of people that like our children having drugs.”
They may ask, “What if we refuse to give our child medications?”

Answer: It is fortunate that most parents have a natural reluctance to give their children medications.
However, it is also true that parents do not want their children to suffer from arthritis, or to suffer
unnecessarily from pain or from joint damage. Sometimes medications are necessary, and they are only
given if it is considered they are less damaging than the condition being treated i.e. arthritis. Parents
need to think about this aspect and consider whether it is fair for a child to have medications withheld
and risk having more damage or pain than necessary. Medications in correct dosages can make life
considerably easier for a child with arthritis, by reducing pain and stiffness, making physiotherapy less
painful and helping to prevent joint damage. However, all medications have potential side effects, and it
is important that parents are aware of these.

11. Question: How important are splints for my child’s joints, and what are they?

Answer: Splinting is very important for certain joints when arthritis is present. For an arthritic joint, the
“position of comfort” or “position of rest” is usually flexed i.e. bent. This means that the particular joint
may be in the flexed position for a large proportion of the time. A joint with arthritis can lose some of the
range of movement if it is not moved through the range regularly, as for a normal joint. Splints are
devices made of light but strong supportive material that are placed next to a joint to hold it in a certain
position and are usually held in place with “velcro”. They are designed to maintain a good range of
movement of a joint, by resting the joint in a functional position and encouraging the use of the full range
of possible movement. When joint range is lost through arthritis, this range can be regained for some
joints, by hard work, splinting and exercises. However, it is not always possible, and it is much better if
the movement of the joints can be maintained from the beginning. Sometimes, in severe arthritis, a joint
may lose most of its range of movement. If this joint is in a good functional position, it may not matter,
and the joint can be functional. Splints are helpful in ensuring that joints at risk, are still able to achieve
the most important part of the range of movement. It is sad to see a child’s joint stuck in a non-functional
position, especially when this did not need to happen to that child.

Knees are the most commonly splinted joints in children with arthritis. The children usually wear a light
removable leg splint while they sleep. Often they become very attached to their nighttime splints.
However, not all knees with arthritis need to be splinted if there is no evidence that the range of
movement is being lessened due to arthritis. Some joints can not be splinted easily e.g. shoulders and
hips.
12. Question: Will my child be able to be a normal independent adult or will my child require on-going
support into adult life?

Answer: It is reasonable to assume that your child will be entirely independent and essentially have a
normal adult time. The majority of children will recover from JIA, and most will lead normal lives. It is not
in a child’s or a teenager’s interest for them to be given the idea that they will be on social benefits later
in life because of childhood arthritis. For most it is out of the question that they would need such support.

13. Question: Is it useful for my child to speak with other children who also have juvenile idiopathic
arthritis?

Answer: Most children and their parents find it helpful to talk with others who are in a similar situation. It
can put the matter into better perspective and it lessens anxiety. It is particularly helpful for children to
see that arthritis in most other children makes little difference to who they are, how they look, what they
can do and what their goals and dreams are.

This booklet was written by Dr. Prudence Manners MD

This page was updated on the  30th January, 2014


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