Pediatrics Dr.

Sawsan Ali

"Juvenile Idiopathic Arthritis"

JIA is the most common rheumatic disease in childhood and a major cause of chronic
disability.

Epidemiology:
Oligo-arthritis is the most common subtype (40-50%), followed by polyarthritis
(25-30%) and systemic JIA (5-15%).
There is no sex predominance in systemic JIA (sJIA), but more girls than boys are
affected in both oligo-articular (3:1) and polyarticular (5:1) JIA.
The peak age at onset is between 2 and 4 yr. for oligoarticular disease. Age of onset
has a bimodal distribution in polyarthritis, with peaks at 2-4 yr. and 10-14 yr. sJIA occurs
throughout childhood with a peak between 1 and 5 yr.

Etiology:

Unknown, but may be due to immune-genetic susceptibility with an external trigger.

JIA is a complex genetic trait in which multiple genes may affect disease susceptibility.
Possible non-genetic triggers include bacterial and viral infections, enhanced immune
responses to bacterial or mycobacterial heat shock proteins, abnormal reproductive
hormone levels, and joint trauma.

Pathogenesis:

JIA is an autoimmune disease associated with infiltration of mononuclear cells in

the affected joint →villous hypertrophy & hyperplasia with hyperemia & edema of
synovial tissue. Advanced uncontrolled disease leads to progressive erosion of articular
cartilage and bone.

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Clinical manifestations:

These are the criteria of American academy of rheumatology

 Age at onset: <16 yr.
 Arthritis (swelling or effusion, or the presence of 2 or more of the following signs:
limitation of range of motion, tenderness or pain on motion, increased heat) in ≥1
joint
 Duration of disease: ≥6 wk.
 Onset type defined by type of articular involvement in the 1st 6 mo. after onset:
 Polyarthritis: ≥5 inflamed joints
 Oligoarthritis: ≤4 inflamed joints
 Systemic-onset disease: arthritis with rash and a characteristic quotidian fever
 Exclusion of other forms of juvenile arthritis.

Initial symptoms may be subtle or acute: morning stiffness with limp or gelling after
inactivity with easy fatigability and poor sleep quality. Involved joints are often: swollen,
warm, painful on movement or palpation with reduced range of motion but usually not
erythematous.

Oligoarthritis: ≤4 inflamed joints during the 1st 6 months of the disease, and often only a
single joint is involved. It predominantly affects the large joints of the lower extremities
e.g. knees and ankles, hip is rare.

Polyarthritis: ≥5 inflamed joints, affect both upper and lower extremities. Micrognathia
may occur and reflects chronic TM joint disease. Cervical spine involvement manifested
as ↓ neck extension, with the risk of atlantoaxial subluxation and neurologic sequelae.

Systemic onset JIA: Systemic manifestations in addition to arthritis e.g. fever, HSM, LAP,
and serositis (pericarditis), present as FUO. The fever is ≥39 C & spiking, especially in
evening, for at least 2 wk.; it is accompanied by faint, erythematous, macular rash "Salmon-
rash" which is non-pruritic, migratory, & lasting <1 hr.

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DIAGNOSIS:
JIA is a clinical diagnosis without any diagnostic laboratory tests. Laboratory
studies, including tests for ANA and RF, are only supportive or prognostic, and their results
may be normal in patients with JIA.

Investigations:

 X-ray of joints in early disease shows soft tissue swelling, periarticular osteoporosis
and periosteal new-bone apposition. Continued active disease may cause
subchondral erosions & loss of cartilage with bony destruction.
 MRI is more sensitive to early changes than radiography.
 CBP show anemia of chronic disease, leukocytosis, & thrombocytosis.
 Inflammatory markers are ↑e.g. ESR, CRP
 ANA is +ve in 40-85% of patients with oligo-& polyarticular arthritis; it is
associated with ↑risk for chronic uveitis
 RF is +ve in only 5-10% of patients with polyarticular arthritis which indicate a bad
prognosis
 Anti–Cyclic Citrullinated peptide (CCP) antibody; it is similar to RF in that it is a
marker of more aggressive disease.

Treatment:
The goals of treatment are to achieve disease remission, prevent or halt joint damage,
and foster normal growth and development. The following medications are used:

 NSAI agents e.g. Naproxen, Ibuprofen, and Meloxicam; S/E: Gastritis, renal
and hepatic toxicity.
 Intra-articular injection of Corticosteroids
 Disease-Modifying Antirheumatic Drugs: such as Methotrexate (which may
take 6-12 wk. for its effects), S/E: Nausea, vomiting, oral ulcerations,
hepatotoxicity, blood count dyscrasias, immunosuppression, and teratogenicity.
Other agents include sulfasalazine and leflunomide.
 Systemic corticosteroids may be recommended for management of severe
systemic illness or for control of uveitis (periodic slit lamp ophthalmologic
examination of all patients is required to monitor asymptomatic uveitis).

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  Biological agents are used to treat children with an inadequate response to
methotrexate, with poor prognostic factors, or with severe disease onset.
Biologic medications involve:
1. Anti–Tumor Necrosis Factor-α (Etanercept, infliximab); S/E:
Immunosuppressant, concern for malignancy, demyelinating disease,
lupus-like reaction, injection site reaction.
2. Anti-CD20: Rituximab.
3. Interleukin-1 Inhibitors: Anakinra.
4. Interleukin-6 Receptor Antagonist: Tocilizumab.
 Dietary therapy includes: adequate intake of calcium, vitamin D, protein, and
calories.

Note: Oligoarthritis is usually responding to NSAIs & IAI of corticosteroids, whereas

Polyarthritis & Systemic-onset diseases are usually required MTX & other agents.

Prognosis:

 Children with oligoarticular disease especially girls with age at onset <6 years are
at risk to develop chronic uveitis.
 The child with polyarticular disease often has a more prolonged course of active
joint inflammation which requires early and aggressive therapy. Predictors of severe
and persistent disease include: young age at onset, presence of RF or anti-CCP
antibodies and large numbers of affected joints.
 Systemic-onset disease is often the most difficult to control in both articular
inflammation and systemic manifestations. Poorer prognosis is related to
polyarticular distribution of arthritis, fever lasting >3 months, and increased
inflammatory markers (e.g. platelet count and ESR) for >6 months.

*** Good Luck ***