Juvenile Idiopathic Arthritis Signs, Types & Physical Exam

Juvenile Idiopathic Arthritis
Prof Ariyanto Harsono MD PhD SpA(K)

Introduction
Juvenile rheumatoid arthritis (JRA), is the most
common chronic rheumatologic disease in
children and is one of the most common chronic
diseases of childhood. The etiology is unknown,
and the genetic component is complex, making
clear distinctions between the various subtypes
difficult. A new nomenclature, juvenile idiopathic
arthritis (JIA), is being increasingly used to
provide better definition of subgroups.
Prof Ariyanto Harsono MD PhD SpA(K) 2

Criteria and classification
Three groups have developed sets of criteria to classify
children with arthritis: the American College of
Rheumatology (ACR), the European League Against
Rheumatism (EULAR), and the International League of
Associations for Rheumatology (ILAR).
The ACR criteria define juvenile rheumatoid arthritis
(JRA) by age limit (< 16 y) and the duration of disease
(>6 weeks). The organization recognizes the following
3 subtypes:
Polyarticular
Pauciarticular
Systemic

Classification ACR 1977 ILAR 1997
Nomenclature Juvenile Rheumatoid Arthritis Juvenile Idiopathic Arthritis
Minimum Duration >6wk >6wk
Age of onset <16yr <16yr
≤ 4 joints in first 6 mo after
presentation
Pauciarticular juvenile rheumatoid arthritis Oligoarticular juvenile idiopathic arthritis:
(A) Persistent < 4 joints for course of disease;
(B) Extended >4 joints after 6 mo
>4 joints in first 6 mo after
presentation
Polyarticular juvenile rheumatoid arthritis Polyarticular juvenile idiopathic arthritis-
rheumatoid factor negative
Polyarticular juvenile arthritis-rheumatoid
factor positive
Fever, rash, arthritis Systemic juvenile rheumatoid arthritis Systemic juvenile idiopathic arthritis
Other categories included Exclusion of other forms Psoriatic juvenile idiopathic arthritis
Enthesitis-related arthritis
Undifferentiated:
(A) Fits no other category;
(B) Fits more than 1 category
Inclusion of psoriatic arthritis,
inflammatory bowel disease, juvenile
ankylosing spondylitis
No Yes

Signs and symptoms
History findings in children with JIA may include the following:
 Arthritis present for at least 6 weeks before diagnosis
(mandatory for diagnosis of JIA)
 Either insidious or abrupt disease onset, often with
morning stiffness or gelling phenomenon and arthralgia
during the day
 Complaints of joint pain or abnormal joint use
 History of school absences or limited ability to participate
in physical education classes
 Spiking fevers occurring once or twice each day at about
the same time of day
 Evanescent rash on the trunk and extremities
 Psoriasis or more subtle dermatologic manifestations

Physical findings are important to provide criteria for
diagnosis and to detect abnormalities suggestive of
alternative etiologies, as well as to indicate disease
subtypes. Such findings include the following:
Arthritis: Defined either as intra-articular swelling on
examination or as limitation of joint motion in
association with pain, warmth, or erythema of the
joint; physical findings in JIA reflect the extent of joint
involvement
Synovitis: Characterized by synovial proliferation and
increased joint volume; the joint is held in a position
of maximum comfort, and range of motion often is
limited only at the extremes

Types of JIA include the following:
Systemic-onset juvenile idiopathic arthritis
Oligoarticular juvenile idiopathic arthritis
Polyarticular juvenile idiopathic arthritis
Psoriatic arthritis
Enthesitis-related arthritis
Undifferentiated arthritis

Arthritis must be present for 6 weeks before the
diagnosis of juvenile idiopathic arthritis (JIA) can
be made. Disease onset is either insidious or
abrupt, with morning stiffness or gelling
phenomenon (ie, stiffness after long periods of
sitting or inactivity) being a frequent complaint
and arthralgia occurring during the day. A morning
limp that improves with time may be noted, and a
toddler may no longer stand in the crib in the
morning or after naps.

Complaints of joint pain may not be predominant in
the patients’ history, however; children often stop
using joints normally (eg, develop contractures of
joints, decreased wrist range, limp) rather than
complain of pain. Up to a quarter of children with
oligoarticular JIA have no pain.
Individuals with JIA may have a history of school
absences, and their ability to participate in
physical education classes reflects the severity of
the disease or acute flares.

Systemic-onset JIA is characterized by spiking fevers,
typically occurring once or twice each day, at about
the same time of day, with temperature returning to
normal or below normal. The fever pattern is very
useful because infections, Kawasaki disease, and
malignancy usually do not have such a predictable
pattern.
Systemic-onset JIA is usually accompanied by an
evanescent rash (lasting a few hours), which is
typically nonpruritic, macular, and salmon colored on
the trunk and extremities. Occasionally, the rash is
extremely pruritic and resistant to antihistamine
treatment.

Children with psoriatic arthritis may have typical
psoriasis but dermatological manifestations may
be subtle; careful attention should be paid to
looking for nail pits. Dactylitis is characteristic of
psoriatic arthritis.
Enthesitis-related arthritis frequently presents as
evening and post-exercise pain. Attention should
be given to buttock pain and back pain that
improves with activity (inflammatory back pain).
These children cannot lie in bed all morning but
have to get up due to back pain.

Physical Examination
JIA is a clinical diagnosis. A complete physical
examination is critical for the diagnosis. Physical
findings are important to provide criteria for
diagnosis and to detect abnormalities suggestive
of alternative etiologies. The diagnosis of JIA is
based on the physical finding of arthritis in at
least 1 joint that has persisted for at least 6
weeks, with other causes excluded, in an
individual younger than 16 years.

No diagnostic serologic tests for JIA are recognized, aside
from rheumatoid factor assay for subclassification of
polyarticular disease. Other tests, such as antinuclear
antibody and HLA-B27 assays, may help further define
diagnosis and risk of complications.
Arthritis is defined as either intra-articular swelling on
examination or as limitation of joint motion in
association with pain, warmth, or erythema of the
joint. The hips, temporomandibular joint, and small
joints in the spine do not demonstrate swelling when
affected by synovitis but demonstrate the combination
of loss of motion and pain. The physical findings in JIA
are a reflection of the extent of joint involvement.

In synovitis, in which there is synovial proliferation
and an increase in joint volume, the joint is held in
a position of maximum comfort. Limbs with
synovitis are generally held in flexion. Range of
motion often is limited only at the extremes.
In synovitis, the fingers may appear swollen, and the
range of motion becomes painful. The wrist goes
into flexion. In the knee, the parapatellar fossae
often are obliterated, and a doughy synovium may
be palpable. A soft, boggy swelling is appreciated
in the popliteal fossa.

The hip is held in an attitude of flexion, abduction,
and external rotation. Attempted range of motion
will be painful to a varying degree. Guarding is an
early sign of synovitis.
Cutaneous erythema is extremely rare in JIA. Its
presence should alert one to look for another
diagnosis.

Systemic-onset JIA
A definite diagnosis of systemic-onset JIA must
await the development of arthritis. This may occur
at onset of the fever and rash or may lag by months
or, rarely, years.

Physical examination findings include the
following:
 The child appears
systemically ill
 Arthralgia is often present
 The child may have
generalized myalgia
 Evanescent, salmon-pink,
macular rash (often linear)
is found, predominantly on
the trunk and the
extremities; this rash, seen
in the image below, is
associated with fever spikes
Systemic juvenile idiopathic arthritis (JIA) rash

 Hepatosplenomegaly is often
present
 Lymphadenopathy is
sometimes present, especially
the axillary lymph nodes
 Muscle tenderness to palpation
may be observed
 Serositis, including pleural and
pericardial effusions, may be
present, as is noted in the
image below Child with pericardial effusion due to
systemic onset juvenile idiopathic arthritis

Chest pain or shortness of breath may be a sign of
pericarditis or pleuritis
Friction rub may occur in pericarditis but can be
absent with a large pericardial effusion
S3, basilar rales, and hepatomegaly suggestive of
heart failure may rarely be observed when
myocarditis occurs in individuals with systemic-
onset JIA

Oligoarticular JIA
Characteristics of oligoarticular
JIA include the following:
 In individuals with
oligoarticular JIA, 4 or fewer
joints (and in many cases,
only 1 joint) are affected
 Large, weight-bearing joints,
such as the knees and
ankles, are typically affected,
as seen in the
Eighteen-month-old girl with arthritis in her
right knee. Note the flexion

Children appear to be well, despite ambulating
with a limp
In cases of asymmetrical arthritis, chronic
inflammation-related hyperemia in a knee or
ankle may lead to overgrowth of that limb with
subsequent leg-length discrepancy

 Muscle atrophy, often of
extensor muscles (eg, vastus
lateralis, quadriceps when the
knee is affected), may occur
 Flexion contractures in the
knees and, less commonly, the
wrists are found
 Involvement of a few small
joints in the hands is atypical
and suggests eventual
development of polyarticular JIA
or psoriatic arthritis. Dactylitis,
or diffuse tenosynovitis of a
finger or toe, also called a
"sausage digit," is more typical
of psoriatic arthritis or
enthesitis-related arthritis.

Anterior uveitis (seen in the image below) is present in
as many as 20% of children with oligoarticular and
polyarticular JIA, especially those who are antinuclear
antibody (ANA) positive. The uveitis is typically
asymptomatic at onset and must be screened for with
an ophthalmologic slit lamp examination.
Generally, children who were 6 years of age or younger
at onset (especially of oligoarticular and psoriatic
arthritis) and have a positive ANA test are screened by
slit lamp exam every 3 months for 4 years or more and
then every 6 months until at least 7 years after
diagnosis. Thereafter they are screened yearly for life.

Children who are at lesser risk (ie, have polyarticular
disease and are ANA negative), are screened every 6
months for 7 years and then yearly. Children with
systemic JIA are at very low risk and are screened
yearly.
Acute anterior uveitis is one of the diagnostic criteria
for enthesitis-related arthritis. These children with are
screened initially and if symptomatic.
Older children with RF-positive polyarticular JIA
should probably be screened yearly. There are few
data on these children regarding their risk for uveitis.

Sequelae of chronic anterior uveitis. Note the posterior synechiae (weblike
attachments of the pupillary margin to the anterior lens capsule) of the right eye
secondary to chronic anterior uveitis. This patient has a positive antinuclear antibodies
(ANAs) and initially had a pauciarticular course of her arthritis. She now has
polyarticular involvement but no active uveitis.

Polyarticular Juvenile Idiopathic
Arthritis
In polyarticular juvenile idiopathic arthritis, 5 or
more joints are affected in the first 6 months after
disease onset, weight-bearing joints are affected,
rheumatoid nodules may be seen in patients with
RF-positive disease, and symmetrical involvement
of small joints in the hands is often found, as seen
in the images below.

Patient with active polyarticular arthritis. Note swelling (effusions) of all proximal
interphalangeal (PIP) joints in addition to boney overgrowth. Also note lack of distal
interphalangeal joint (DIP) involvement. The patient has interosseus muscle wasting
(observed on the dorsum of the hands), and subluxation and ulnar deviation of the
wrists are present.

Decreased extension of the cervical spine is often asymptomatic. It is indicative of
arthritis of the cervical spine and can lead to subluxation, typically of the C2 vertebra
on C3. Fusion of the posterior elements of the vertebra may occur.
Flexion and extension views of C-spine in child with poorly controlled
polyarticular juvenile idiopathic arthritis (JIA).

Psoriatic Arthritis
Psoriatic arthritis in children is usually mild. Onset of arthritis
precedes that of psoriasis in approximately half of children.
 Characteristics of psoriatic arthritis include the following:
 Monoarticular arthritis (50% of children)
 DIP joint involvement (50%)
 Tenosynovitis (30%)
 Nail involvement(71%) - pitting is the most common but least
specific finding
 Disordered bone growth with resultant shortening (47%)
 Sacroiliitis (28%)

Enthesitis-Related Arthritis
Enthesitis-related arthritis, or pediatric
spondyloarthropathy, is characterized by periods of
inflammation of tendons and ligaments, particularly
at the area of insertion into bone (entheses). Often,
children and adolescents with spondyloarthropathy
present with arthritis, making the distinction
between subtypes difficult. Furthermore, children
occasionally develop a disease that appears to be a
combination of the 2 diseases.

Pain and tenderness at the enthesis is the most
common manifestation, but swelling may also be
seen. In children, the initial manifestations involve
mainly the peripheral joints (eg, dactylitis) with
asymmetric oligoarticular arthritis of the lower
limbs; axial involvement (eg, sacroiliitis) tends to
appear later in the disease course.

Diagnostic criteria for enthesitis-related JIA are the presence of
both arthritis and enthesitis, or the presence of arthritis or
enthesitis along with any 2 of the following 5 manifestations:
 Sacroiliac tenderness and/or inflammatory lumbosacral pain
 Positive human leukocyte antigen B27 (HLA-B27) test
 Onset of arthritis in a male 6 years old or older
 Acute symptomatic anterior uveitis
 Presence in a first-degree relative of ankylosing spondylitis,
enthesitis-related arthritis, inflammatory bowel disease with
sacroiliitis, reactive arthritis, or acute anterior uveitis
 Although enthesitis can be observed in persons with
oligoarticular and polyarticular JIA, the eventual development
of arthritis into a predominant enthesitis is more characteristic
of spondyloarthropathy. The radiographic changes observed in
adults (eg, sclerosis of the sacroiliac joints, bamboo spine) are
rare in childhood and adolescence.

Diagnosis
Diagnosis of JIA is based on the history and physical examination findings.
When physical findings do not document definite arthritis, further evaluation
is warranted. However, laboratory studies help to exclude other underlying
disorders, classify the type of arthritis, and evaluate for extra-articular
manifestations of JIA. Laboratory studies that may be considered include the
following:
 Inflammatory markers: Erythrocyte sedimentation rate (ESR) or CRP level
 Complete blood count (CBC) and metabolic panel
 Liver function tests and assessment of renal function with serum creatinine
levels
 Antinuclear antibody (ANA) testing
 Rheumatoid factor (RF) and anti–cyclic citrullinated peptide (CCP) antibody
 Additional studies: Total protein, albumin, fibrinogen, ferritin, D-dimer,
angiotensin-converting enzyme (ACE), antistreptolysin 0 (AS0), anti-DNAse
B, urinalysis
 When only a single joint is affected, radiography is important to exclude
other diseases. Basic radiographic changes in JIA include the following:

o Soft tissue swelling
o Osteopenia or osteoporosis
o Joint-space narrowing
o Bony erosions
o Intra-articular bony ankylosis
o Periosteitis
o Growth disturbances
o Epiphyseal compression fracture
o Joint subluxation
o Synovial cysts

Inflammatory Markers
The erythrocyte sedimentation rate (ESR) or C-
reactive protein (CRP) level is usually elevated in
children with systemic-onset JIA (with a
disproportionate increase in the CRP) and may be
elevated in those with polyarticular disease; however,
it is often within the reference range in those with
oligoarticular disease. When elevated, inflammatory
markers can be used to monitor disease activity.
Other markers of inflammation include
thrombocytosis, leukocytosis, complement, and, in a
reverse fashion, albumin and hemoglobin.

Lymphopenia is not uncommon because of
emigration of activated lymphocytes out of the
circulation into synovium. However, neutropenia is
uncommon and, particularly with lymphocytosis or
thrombocytopenia, raises the possibility of acute
lymphocytic leukemia.

Complete Blood Count and Metabolic Panel
A complete blood count, liver function tests (to
exclude the possibility of viral or autoimmune
hepatitis), and assessment of renal function with
serum creatinine levels should be done before
starting treatment with nonsteroidal anti-
inflammatory drugs (NSAIDs), methotrexate (MTX),
or tumor necrosis factor–alpha inhibitors.

Antinuclear Antibody Testing
As many as 70% of children with oligoarticular JIA
have positive ANA assays. However, a positive ANA
should also raise suspicion of SLE. Overlap between
the manifestations of the two disorders may lead to
initial misdiagnosis of SLE as JIA.
A positive ANA is a marker for increased risk of
anterior uveitis. Children younger than 6 years at
arthritis onset with a positive ANA finding are in the
highest risk category for development of uveitis and
need slit lamp screening every 3-4 months. Titers do
not correlate with disease activity.

Radiography
When only a single joint is affected, radiography is important
to exclude other diseases, such as osteomyelitis. Basic
radiographic changes in JIA (see the images below) include
the following:
 Soft tissue swelling
 Osteopenia and/or osteoporosis
 Joint-space narrowing
 Bony erosions
 Intra-articular bony ankylosis
 Periosteitis
 Growth disturbances
 Epiphyseal compression fracture
 Joint subluxation

Ankylosis in the cervical spine
at several levels due to long-
standing juvenile rheumatoid
arthritis (also known as
juvenile idiopathic arthritis).

Widespread osteopenia, carpal
crowding (due to cartilage loss),
and several erosions affecting the
carpal bones and metacarpal
heads in particular in a child with
advanced juvenile rheumatoid
arthritis (also known as juvenile
idiopathic arthritis).
The main limitation of
conventional radiography is that it
does not allow direct examination
of the articular cartilage,
synovium, and other important
noncalcified structures in a joint.

Computed Tomography and Magnetic
Resonance Imaging
CT scanning is the best method for analyzing bony
abnormalities, but it has been largely superseded by
MRI in the overall assessment of JIA. The major
disadvantage of CT scanning is that it involves a
substantial radiation dose. Perform CT scanning of the
long bones when considering osteoid osteoma is
suspected.
MRI is helpful when considering trauma in the
differential diagnosis. In addition, imaging of the TMJ,
sacroiliac joint, cervical spine, midfoot, hip, or
shoulder is useful in diagnosing inflammatory
arthritis. Prof Ariyanto Harsono MD PhD SpA(K) 42

(A) T2-weighted MRI shows high
signal in both hips, which may be
due to hip effusions or synovitis.
High signal intensity in the left
femoral head indicates avascular
necrosis.
(B) Coronal fat-saturated
gadolinium-enhanced T1-weighted
MRI shows bilateral enhancement
in the hips. This indicated bilateral
active synovitis, which is most
pronounced on the right. Because
the image was obtained with fat
saturation, the hyperintensity in
both hips is pathologic, reflecting
an inflamed pannus.

MRI provides the most sensitive radiologic indicator of disease
activity. The modality can depict synovial hypertrophy, define
soft tissue swelling, and demonstrate excellent detail of the
status of articular cartilage and overall joint integrity.
To improve visualization of synovial hypertrophy and improve
detection of cartilaginous erosions when an inflammatory
arthritis is suspected, contrast-enhanced sequences should be
performed.
Synovitis and a joint effusion may have similar hyperintensity
on T2-weighted (T2W) and short-tau inversion recovery (STIR)
images. Therefore, gadolinium-enhanced T1-weighted (T1W)
MRIs are necessary to accurately define active synovitis.
Note that gadolinium-based contrast agents (gadopentetate
dimeglumine [Magnevist], gadobenate dimeglumine
[MultiHance], gadodiamide [Omniscan], gadoversetamide
[OptiMARK], gadoteridol [ProHance]) have been linked to the
development of nephrogenic systemic fibrosis (NSF) or
nephrogenic fibrosing dermopathy (NFD).Prof Ariyanto Harsono MD PhD SpA(K) 44

Ultrasonography
On ultrasonograms, inflamed synovium can appear as an area
of mixed echogenicity lining the articular cartilage; the
vascularity of the synovium can be assessed with Doppler
flow studies. Serial measurements of synovial thickness and
effusion volumes have been used to monitor disease
progression.[28] It can be helpful to evaluate joints that are
difficult to palpate, such as the hip and shoulder.
Some researchers claim that ultrasonography is more
sensitive than plain radiography in the detection of cartilage
erosions and effusions. Ultrasound has the advantages of no
exposure to ionizing radiation; it can be done in the clinic is an
awake, moving child; and it can help guide injections.

Pathogenesis
The etiology and pathogenesis of JIA are not completely
understood. Genetic susceptibility plays a major role, but
there is significant overlap between loci associated with JIA
and those associated with other autoimmune diseases.
JIA is a genetically complex disorder in which multiple genes
are important for disease onset and manifestations.
The IL2RA/CD25 gene has been implicated as a JIA
susceptibility locus, as has the VTCN1 gene. Associations
have been found between specific HLA alleles and clinical
subtypes of JIA (eg, HLA-A(*)02:06 with susceptibility to JIA
accompanied by uveitis, and HLA-DRB1(*)04:05 with
polyarticular JIA, in a Japanese cohort).

Humoral and cell-mediated immunity are involved in the
pathogenesis of JIA. T lymphocytes have a central role,
releasing proinflammatory cytokines (eg, tumor
necrosis factor–alpha [TNF-α], interleukin [IL]-6, IL-1)
and favoring a type-1 helper T-lymphocyte response. A
disordered interaction between type 1 and type 2 T-
helper cells has been postulated.
Studies of T-cell receptor expression confirm recruitment
of T-lymphocytes specific for synovial nonself
antigens. Evidence for abnormalities in the humoral
immune system include the increased presence of
autoantibodies (especially antinuclear antibodies),
increased serum immunoglobulins, the presence of
circulating immune complexes, and complement
activation.

Chronic inflammation of synovium is characterized
by B-lymphocyte infiltration and expansion.
Macrophages and T-cell invasion are associated
with the release of cytokines, which evoke
synoviocyte proliferation. A study by Scola et al
found synovium to contain messenger ribonucleic
acid (mRNA) for vascular endothelial growth factor
and angiopoietin 1, as well as for their receptors,
suggesting that induction of angiogenesis by
products of lymphocytic infiltration may be
involved in persistence of disease.

Some pediatric rheumatologists view systemic-onset JIA as an
autoinflammatory disorder, such as familial Mediterranean
fever (FMF) or cryopyrin-associated periodic fever
syndromes, rather than a subtype of JIA. This theory is
supported by work demonstrating similar expression
patterns of a phagocytic protein (S100A12) in systemic-
onset JIA and FMF, as well as the same marked
responsiveness to IL-1 receptor antagonists.
FMF is associated with mutations in the MEFV gene; these
mutations are associated with activation of the IL-1b
pathway, resulting in inflammation. A study by Ayaz et al
found an increased frequency of MEFV mutations in Turkish
children who were diagnosed with systemic JIA; this study
has not been replicated in other populations.

Prognosis
Advances in treatment over the last 20 years—
especially the introduction of early use of intra-
articular steroids, methotrexate, and biologic
medications—have dramatically improved the
prognosis for children with arthritis. Almost all
children with JIA lead productive lives. However,
many patients, particularly those with polyarticular
disease, may have problems with active disease
throughout adulthood, with sustained remission
attained in a minority of patients.

Early hip or wrist involvement, symmetrical disease, the
presence of RF, and prolonged active systemic disease have
been associated with poor long-term outcomes. Compared
with adults with RF-positive rheumatoid arthritis, however,
children are at less risk for rheumatoid lung involvement
and vasculitis. The anti – cyclic citrullinated peptide
antibodies (CCP) antibodies test may be more specific than
the RF test, but it is not as well studied in children.
Children with systemic-onset disease tend to either respond
completely to medical therapy or develop a severe
polyarticular course that tends to be refractory to medical
treatment, with disease persisting into adulthood.
Most children with oligoarticular disease demonstrate
eventual permanent remission, although a small number
progress to persisting polyarticular disease.

Undifferentiated Arthritis
Undifferentiated JIA is diagnosed if the patient’s
manifestations either do not fulfill the criteria for
any one category or fulfill the criteria for more than
one.
Most often, children in the latter category fulfill the
criteria for polyarticular RF-negative JIA and either
enthesitis-related JIA or psoriatic JIA.

Complications of Disease
Systemic-onset juvenile idiopathic arthritis
The complications that may occur in systemic-onset JIA are
pericarditis, hemolytic anemia, macrophage activation syndrome
(MAS), and endarteritis. Patients with pericarditis often present
with orthopnea and respond to intravenous (IV) corticosteroid
treatment.
MAS is a rare, but important, complication, in which all 3
bloodlines become rapidly decreased. Hypofibrinogenemia,
thrombocytopenia, and elevated aspartate aminotransferase levels
and markedly elevated ferritin levels are hallmarks. Hypotension,
central nervous system (CNS) disease, and marked
hepatosplenomegaly may be noted as complications of a release of
massive amounts of cytokines.

Children with limited arthritis
Complications of oligoarticular JIA and psoriatic arthritis include
joint contractures, uveitis, and leg-length discrepancy. Uveitis is
almost always asymptomatic and more frequent in young girls
who have positive levels of antinuclear antibody. Evaluation with a
slit-lamp every 4 months by a pediatric ophthalmologist can
detect early disease to prevent permanent eye damage and even
blindness.
Leg-length discrepancy may complicate unilateral knee
involvement. In young children, it may result from
neovascularization of growth plates, so the involved limb is longer.
In early puberty, unilateral arthritis can lead to premature fusion
of the epiphysis, in which case the short limb is on the affected
side. The problem may not be detected in patients with a knee
flexion contracture until the contracture is corrected. Both flexion
contractures and leg-length discrepancies are much less frequent
with early intervention.

Children with widespread arthritis
Complications of polyarticular JIA include skeletal
abnormalities such as increased size of epiphyses,
accelerated bone age, narrowed joint spaces, swan-
neck and/or boutonniere deformities, joint subluxation,
and cervical spine involvement.
Difficulty extending the spine may create a problem for
intubation prior to surgery, so anesthesiologists need to
be informed of the patient's diagnosis. Cervical spine
radiography (in flexion and extension) may help to
screen for potential difficulties during induction of
anesthesia. High-level subluxation is a potential
complication

Enthesitis-related arthritis
Complications of enthesitis-elated arthritis are rare
but can include restrictive lung disease and aortic
insufficiency. These children are at risk for
symptomatic iritis with acute photophobia and
conjunctivitis but only rarely does it lead to visual
impairment.

Differential Diagnosis
• Behcet Syndrome
• Infectious Mononucleosis
• Kawasaki Disease
• Lyme Disease
• Pediatric Acute Lymphoblastic Leukemia
• Pediatric Kawasaki Disease
• Pediatric Sarcoidosis
• Pericarditis, Viral
• Somatoform Disorder: Pain
• Systemic Lupus Erythematosus

Differential Diagnosis of Arthritis

Treatment
The ultimate goals in managing rheumatoid arthritis are to
prevent or control joint damage, to prevent loss of function,
and to decrease pain. These goals are particularly important in
juvenile idiopathic arthritis (JIA), in which the rate of
progression and the onset of debility can be rapid. JIA is a
chronic disease characterized by periods of remission and
flare. Treatment is aimed at inducing remission with the least
toxicity from medications with hopes of inducing a permanent
remission.
The success of therapy is monitored best with repeated
physical examinations and history. The number of joints
involved and the duration of morning stiffness should
demonstrate continued decrease, with elimination reflecting
success. Surgery may be indicated in patients who are
unresponsive to medical therapy.

A team-based approach to the treatment of JIA can
be helpful. Management may include 1 or all of the
following areas:
Pharmacologic therapy with nonsteroidal anti-
inflammatory drugs (NSAIDs), disease-modifying
antirheumatic drugs (DMARDs), biologic agents, or
intra-articular and oral corticosteroids
Psychosocial interventions
Measures to enhance school performance (eg,
academic counseling)
Improved nutrition
Physical therapy
Occupational therapy

American College of Rheumatology (ACR) criteria for
complete remission are as follows:
No inflammatory joint pain
No morning stiffness
No fatigue
No synovitis
No progression of damage, as determined in
sequential radiographic examinations
No elevation of the erythrocyte sedimentation rate
(ESR) and C-reactive protein (CRP) levels

The ACR issued recommendations for the
treatment of JIA on the basis of the following 5
treatment groups:
History of arthritis in 4 or fewer joints
History of arthritis in 5 or more joints
Active sacroiliac arthritis
Systemic arthritis without active arthritis
Systemic arthritis with active arthritis

History of arthritis in 4 or fewer jointsAccording to ACR guidelines, the treatment group that comprises patients
who have developed active arthritis in only 4 or fewer joints total
throughout their disease course includes patients in the International
League of Associations for Rheumatology (ILAR) categories of persistent
oligoarthritis, as well as patients with psoriatic arthritis, enthesitis-related
arthritis, and undifferentiated arthritis.
In this treatment group, escalation of therapy typically proceeds from
NSAIDs to intra-articular glucocorticoid injections to methotrexate to TNF-α
inhibitors.
NSAIDs alone may be adequate for patients with involvement of a single
joint and other indications of low disease activity (eg, normal inflammatory
marker levels); response should be evident within 2 months. For other
patients, NSAIDs may be used as adjunctive treatment, as needed.
Intra-articular injections of triamcinolone can be used for any joint involved
with active arthritis, and should provide clinical relief for at least 4 months.
If so, the injections can be repeated as needed.

Methotrexate can be instituted in patients who fail to show
adequate response to NSAIDs and/or joint injections.
Alternatively, methotrexate is recommended as initial
treatment for patients in this treatment group who have high
disease activity and features indicating poor prognosis. In
patients with enthesitis-related JIA, sulfasalazine rather than
methotrexate is recommended for patients who have an
inadequate response to joint injection or an adequate trial of
NSAIDs.
Patients in this treatment group who fail to respond
adequately to joint injections and to 3-6 months (depending
on disease characteristics and severity) of methotrexate are
candidates for TNF-α treatment. The same is true of patients
with enthesitis-related JIA who receive sulfasalazine.

History of Arthritis in 5 or More Joints
This group comprises patients who have
developed active arthritis in 5 or more joints total
throughout throughout their disease course.
Patients need not currently have active
involvement in 5 or more joints. According to ACR
guidelines, this group includes patients with the
ILAR categories of extended oligoarthritis,
rheumatoid factor (RF) negative and RF-positive
polyarthritis, psoriatic arthritis, enthesitis-related
arthritis, and undifferentiated arthritis.

Treatment in this group places less emphasis on initial NSAIDs.
After 1 month of NSAID treatment in patients with low
disease activity, or 1-2 months in those with moderate disease
activity but without poor prognostic features (ie, hip or
cervical spine involvement, positive RF or anti-cyclic
citrullinated peptide antibodies (CCP), radiographic signs of
joint damage), it is appropriate to escalate to methotrexate,
plus adjunctive NSAIDs and joint injection as needed.
In patients with moderate disease activity and poor
prognostic features, as well as in patients with high disease
activity, treatment may start with methotrexate.
Leflunomide may be used as an alternative to methotrexate,
after a failed NSAID trial, or as initial treatment in patients
with high disease activity and poor prognostic features.

The US Food and Drug Administration (FDA) has approved the
interleukin (IL)-6 inhibitor tocilizumab for the treatment of
polyarticular JIA in children 2 years of age and older with
active disease. Tocilizumab can be used alone or in
combination with methotrexate.
Escalation to a TNF-α inhibitor follows if 3-6 months
(depending on disease characteristics and severity) of
methotrexate or leflunomide provides inadequate control.
Patients who show inadequate response after 3-4 months
(depending on disease characteristics and severity) of TNF-α
inhibitor treatment can be switched to a different TNF-α
inhibitor or to abatacept. If these agents prove inadequate,
patients may be started on rituximab; this agent may be most
appropriate in patients with RF-positive polyarticular JIA.

Active Sacroiliac Arthritis
This group includes all patients with clinical and
imaging evidence of active sacroiliac arthritis. It may
include patients from any of the ILAR JIA categories.
Use of a TNF-α inhibitor is recommended more
readily for patients in this group. A TNF-α inhibitor
may be started after failure of an adequate trial of
NSAIDs or after 3-6 months (depending on disease
characteristics and severity) of methotrexate or
sulfasalazine proves inadequate.

Systemic Arthritis with Active Systemic
Features and without Active Arthritis
This group includes all patients who fulfill the ILAR
criteria for systemic arthritis and who have active
fever of systemic JIA with or without other systemic
features, but without active arthritis. A 2-week trials
of NSAIDs may be used in patients who have fever
and less severe disease, and have had significant
active systemic disease for less than 6 months; after
that, patients should be started on systemic
glucocorticoids, with adjunct NSAIDs as needed.

Patients with high systemic disease activity (eg,
significant serositis) may be started on steroids as a
first step. There is virtually no published evidence
regarding steroid doses or administration routes in
this setting.
Patients who sustain or develop active fever while
on systemic steroid therapy can be started on
anakinra. This agent may be a first choice in
patients who have had significant active systemic
disease for at least 6 months.

The FDA has approved tocilizumab for the treatment
of systemic JIA. Clinical trials in children with JIA
showed significantly fewer disease flares when
treated with tocilizumab compared with placebo
(26% vs 48%). Additionally, a higher success rate of
steroid reduction/discontinuance was achieved in
the tocilizumab group (24%) compared with placebo
(3%).

Systemic Arthritis with Active Arthritis
and without Active Systemic Features
This category includes all patients who fulfill the ILAR criteria for
systemic arthritis and who have active arthritis, but who do not
have active systemic features. Most of these patients are started
on NSAIDs while their diagnostic assessment progresses.
NSAID therapy, with intra-articular joint injections as needed, may
be adequate for patients with low disease activity who do not
have hip involvement or radiographic signs of joint damage. After
up to 1 month, however, methotrexate can be added for patients
with any degree of disease severity who continue to have active
arthritis.
After 3 months of methotrexate therapy, the next step in
escalation is to anakinra or a TNF-α inhibitor, although etanercept
is less effective in systemic arthritis than in other forms of
JIA. Patients who show inadequate response to TNF-α inhibitor
treatment can be started on abatacept.
72

Treatment of Macrophage Activation
Syndrome
MAS) is a rare but important complication of systemic-
onset JIA in which numbers of all 3 bloodlines become
rapidly decreased. Hypofibrinogenemia,
thrombocytopenia, and elevated aspartate
aminotransferase levels are hallmarks.
MAS often responds to cyclosporin A, and some case
reports have detailed a response to anakinra.
Treatment of MAS is a medical emergency and should
be performed by physicians familiar with this
complication.

Treatment of Uveitis
Uveitis is often asymptomatic. Patients are typically young girls
who have positive levels of ANA.
Treatment with topical corticosteroid medication and with
mydriatic agents (to prevent closed-angle glaucoma) often can
prevent progression of disease to development of calcium
deposition in the lens (band keratopathy) and adhesions of the iris
to the lens (posterior synechiae), in which an irregular pupillary
margin develops. Such complications may herald a chronic active
disease in which vision is threatened.
Immunosuppressive agents, such as methotrexate or cyclosporine,
may help control chronic uveitis. Infliximab can be effective in
some patients who are resistant to immunosuppressive agents.

Long-Term Monitoring
A complete blood cell count and measurement of liver
enzymes and serum creatinine should be part of routine
follow-up in JIA patients. For JIA patients receiving NSAIDs on
a long-term daily basis, these tests, plus urinalysis, should be
done twice yearly; in patients taking these agents 3-4 days
per week, testing should be repeated annually.
In JIA patients taking methotrexate, these tests should be
conducted approximately 1 month after initiation of routine
use and approximately 1-2 months after any increase in dose.
If prior results were normal and the patient is on a stable
dose, the tests can be repeated approximately every 3-4
months.
In JIA patients taking TNF inhibitors, these tests should be
repeated approximately every 3-6 months. Tuberculosis
screening should be repeated approximately once yearly.

Reference
• Sherry DD. Juvenile Idiopathic Arthritis.
http://emedicine.medscape.com/article/1007
276-overview . Accessed Nov 9, 2014.

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Juvenile Idiopathic Arthritis Signs, Types & Physical Exam

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