ADOLESCENT

JUVENILE IDIOPATHIC ARTHRITIS

• A group of rheumatic diseases that begins by age 16
• Arthritis, fever, rash, adenopathy, splenomegaly, and
iridocyclitis are typical of some forms
• Diagnosis is clinical
• Treatment involves intra-articular corticosteroids and
disease-modifying antirheumatic drugs
• Is uncommon
• Cause of JIA is unknown, but there seems to be a genetic
predisposition as well as autoimmune and
autoinflammatory pathophysiology
• Vast majority of children with JIA have a disease that is
distinct from adult rheumatoid arthritis (RA), but in 3 to 5%
of children with JIA, the disease is analogous to adult RA
CLASSIFICATION OF JIA

• Not a single disease

• The term applies to a number of chronic, noninfectious
arthritides that occur in children and share certain
features
• The current classification system, from the International
League of Associations for Rheumatology, defines
categories of disease based on clinical and laboratory
findings
CLASSIFICATION OF JIA

Some of the categories are subdivided into different forms.

Categories include the following:
• Oligoarticular JIA (persistent or extended)
• Polyarticular JIA (rheumatoid factor [RF] negative or
positive)
• Enthesitis-related arthritis
• Psoriatic JIA
• Undifferentiated JIA
• Systemic JIA
CLASSIFICATION OF JIA
• Many of these categories likely include more than one
disease but are useful to help group children with a similar
prognosis and response to treatment
• Also, children sometimes move to different categories
during the course of their illness
• Oligoarticular JIA is the most common form and typically
affects young girls. It is characterized by involvement
of ≤ 4 joints during the first 6 months of disease.
Oligoarticular JIA is further divided into 2 types: persistent
(always ≤ 4 joints involved) and extended (≥ 5 joints
involved after the first 6 months of disease).
CLASSIFICATION OF JIA

• Oligoarticular JIA is the most common form and typically

affects young girls
• Characterized by involvement of ≤ 4 joints during the first 6
months of disease
• Oligoarticular JIA is further divided into 2 types:
Persistent (always ≤ 4 joints involved)
Extended (≥ 5 joints involved after the first 6 months of
disease)
CLASSIFICATION OF JIA
• Polyarticular JIA is the second most common form
• Affects ≥ 5 joints at onset and is divided into 2 types:
RF negative
RF positive
• Typically, young girls are RF negative and have a better
prognosis
• The RF-positive type typically occurs in adolescent girls
and is analogous to adult rheumatoid arthritis
• In both types, arthritis can be symmetric and frequently
involves the small joints
CLASSIFICATION OF JIA

• Enthesitis-related arthritis involves arthritis and enthesitis

(painful inflammation at the insertion of tendons and
ligaments)
• More common among older boys, and these patients
may subsequently develop arthritis of their axial skeleton
(sacroiliac and lumbar spine)
• Enthesitis-related arthritis tends to be in the lower
extremities and asymmetric
CLASSIFICATION OF JIA

• Psoriatic JIA has a bimodal age distribution

• One peak occurs in young girls, and the other peak
occurs in older males and females (who are equally
affected)
• It is associated with psoriasis, dactylitis (swollen digits), nail
pits, or a family history of psoriasis in a 1st-degree relative
• Arthritis is frequently oligoarticular
CLASSIFICATION OF JIA

• Undifferentiated JIA is diagnosed when patients do not

meet criteria for any one category or meet criteria for
more than one
• Systemic JIA (Still disease) involves fever and systemic
manifestations
CLASSIFICATION OF JIA

• Undifferentiated JIA is diagnosed when patients do not

meet criteria for any one category or meet criteria for
more than one
• Systemic JIA (Still disease) involves fever and systemic
manifestations
SIGNS AND SYMPTOMS OF JIA

• Manifestations involve the joints and sometimes the eyes

and/or skin; systemic juvenile idiopathic arthritis may
affect multiple organs
• Children typically have joint stiffness, swelling, effusion,
pain, and tenderness, but some children have no pain
• Joint manifestations may be symmetric or asymmetric,
and involve large and/or small joints
• Enthesitis typically causes tenderness of the iliac crest and
spine, greater trochanter of the femur, patella, tibial
tuberosity, achilles insertion, or plantar fascia insertions
SIGNS AND SYMPTOMS OF JIA

• Sometimes, JIA interferes with growth and development

• Micrognathia (receded chin) due to early closure of
mandibular epiphyses or limb length inequality (usually
the affected limb is longer) may occur
SIGNS AND SYMPTOMS OF JIA

• The most common comorbidity

is iridocyclitis (inflammation of the anterior chamber and
anterior vitreous) that is typically asymptomatic but
sometimes causes blurring of vision and miosis
• Rarely, in enthesitis-related arthritis, there are also the
more common uveitis manifestations of conjunctival
injection, pain, and photophobia
SIGNS AND SYMPTOMS OF JIA

• Iridocyclitis can result in scarring (synechia), cataracts,

glaucoma, or band keratopathy
• Iridocyclitis is most common in oligoarticular JIA,
developing in nearly 20% of patients, especially if patients
are positive for antinuclear antibodies (ANA)It may occur
in the other forms but is exceedingly rare in polyarticular
RF-positive JIA and systemic JIA
SIGNS AND SYMPTOMS OF JIA

• Skin abnormalities are present mainly in psoriatic JIA, in

which psoriatic skin lesions, dactylitis, and/or nail pits may
be present, and in systemic JIA, in which a typical
transient rash often appears with fever
• Rash in systemic JIA may be diffuse and migratory, with
urticarial or macular lesions with central clearing
SIGNS AND SYMPTOMS OF JIA
• Systemic abnormalities in systemic JIA include high fever,
rash, splenomegaly, generalized adenopathy (especially
of the axillary nodes), serositis with pericarditis or pleuritis,
and lung disease
• These symptoms may precede the development of
arthritis
• Fever occurs daily (quotidian) and is often highest in the
afternoon or evening and may recur for weeks
• In 7 to 10% of patients, systemic JIA may be complicated
by macrophage activation syndrome, a life-threatening
cytokine storm syndrome
DIAGNOSIS OF JIA
• Clinical evaluation
• Rheumatoid factor (RF), antinuclear antibodies (ANA),
anticyclic citrullinated peptide (anti-CCP) antibodies, and
HLA-B27 tests
• Juvenile idiopathic arthritis should be suspected in
children with symptoms of arthritis, signs of iridocyclitis,
generalized adenopathy, splenomegaly, or unexplained
rash or prolonged fever, especially if quotidian
• Diagnosis of JIA is primarily clinical, made when a chronic
noninfectious arthritis lasting > 6 weeks has no other
known cause
DIAGNOSIS OF JIA

• Patients with JIA should be tested for RF, anti-CCP

antibodies, ANA, and HLA-B27 because these tests may
be helpful in distinguishing between forms
• The test for ANA should be done by immunofluorescence
because other methods may result in false-negative
results
DIAGNOSIS OF JIA

• In systemic JIA, RF and ANA are usually absent

• In oligoarticular JIA, ANA are present in up to 75% of
patients and RF is usually absent
• In polyarticular JIA, RF usually is negative, but in some
patients, mostly adolescent girls, it can be positive
• HLA-B27 is present more commonly in enthesitis-related
arthritis
DIAGNOSIS OF JIA

• In systemic JIA, laboratory abnormalities suggestive of

systemic inflammation, such as elevated erythrocyte
sedimentation rate (ESR), ferritin, and C-reactive protein,
along with leukocytosis, anemia, and thrombocytosis are
almost always present at diagnosis
DIAGNOSIS OF JIA

• To diagnose iridocyclitis, a slit-lamp examination should

be done even in the absence of ocular symptoms
• A recently diagnosed patient with oligoarticular or
polyarticular JIA should have an eye examination every 3
months if ANA test results are positive and every 6 months
if ANA test results are negative
PROGNOSIS OF JIA

• Remissions occur in 50 to 70% of treated patients

• Patients with RF-positive polyarticular JIA have a less
favorable prognosis
TREATMENT OF JIA
• Drugs that slow disease progression
(particularly methotrexate, tumor necrosis factor [TNF]
inhibitors, and interleukin [IL]-1 inhibitors)
• Intra-articular corticosteroid injections
• Sometimes nonsteroidal anti-inflammatory drugs (NSAIDs)
for symptom relief
• Similar to the therapy of patients with adult rheumatoid
arthritis, disease-modifying antirheumatic drugs (DMARDs),
particularly methotrexate and the biologic agents (eg,
etanercept, anakinra,
canakinumab, tocilizumab, abatacept)
TREATMENT OF JIA

• Methotrexate is useful for oligoarticular, psoriatic, and

polyarticular forms of JIA
• Adverse effects are monitored as in adults
• Bone marrow depression and hepatic toxicity are
monitored with complete blood count, aspartate
aminotransferase (AST), alanine aminotransferase (ALT),
and albumin
• Occasionally, sulfasalazine is used, especially in cases of
suspected spondyloarthritis
TREATMENT OF JIA
• Except for severe systemic disease, systemic
corticosteroids can usually be avoided
• When necessary, the lowest possible dose is used (eg,
range for oral prednisone, 0.0125 to 0.5 mg/kg 4 times a
day, or the same daily dose given once or twice a day
• Growth retardation, osteoporosis, and osteonecrosis are
the major hazards of prolonged corticosteroid use in
children
• Intra-articular corticosteroid injections can be given. The
dosage for children is adjusted based on weight. Some
children may need to be sedated for intra-articular
injection, especially if multiple joints require injection
TREATMENT OF JIA

• Symptoms of juvenile idiopathic arthritis may be reduced

with NSAIDs but they do not alter long-term joint disease
or prevent complications
• NSAIDs are most useful for enthesitis
• Naproxen 5 to 10 mg/kg orally 2 times a day, ibuprofen 5
to 10 mg/kg orally 4 times a day, and indomethacin 0.5 to
1.0 mg/kg orally 3 times a day are among the most useful
TREATMENT OF JIA

• Iridocyclitis is treated with ophthalmic corticosteroid drops

and mydriatics and may require
systemic methotrexate and anti-TNF therapy, and,
occasionally, surgery
• Physical therapy, exercises, splints, and other supportive
measures may help prevent flexion contractures
• Adaptive devices can improve function and minimize
unnecessary stresses on inflamed joints.