Systemic Onset
- Epidemiology: M=F, 1-6yo
- Signs and symptoms:
- High spiking fevers daily or twice daily
- Evanescent salmon-colored rash
- Lymphadenopathy, hepatosplenomegaly, pericarditis
- Labs: RF(-), ANA(-), increased ferritin, leukocytosis, anemia, increased ESR
- If persists: osteoporosis, short stature, brachydactyly, micrognathia
- Differential: infection (bacterial, viral), malignancy, inflammatory bowel disease, acute rheumatic fever, systemic vasculitides (polyarteritis nodosa), collagen vascular disease (SLE)
- Complications: Pericardial tamponade, systemic vasculitis, macrophage activation syndrome (persistent high fever, pancytopenia, active phagocytosis on bone marrow biopsy)
Pauciarticular
- Epidemiology: F>M, 2-3yo, rare <10yo
- Persistent – affects no more than 4 joints after 6mo
- Extended – affects more than 4 joints after 6mo � erosive arthritis, treatment: immunosuppressant
- Signs and symptoms:
- �4 joints especially knee and ankle
- Uveitis
- Labs: ANA(+)
- If persists: leg length discrepancy, eye problems (corneal clouding, cataracts, glaucoma, visual loss)
- Differential: infection (get joint aspiration to rule out), Lyme, postviral, trauma, malignancy, mechanical
Polyarticular
- RF(+)
- Epidemiology: F>M, �8yo, (+)HLA-DR4
- Signs and symptoms: symmetric, small joints
- Complications: severe erosive arthritis, rheumatoid nodules, worse prognosis than RF(-)
- RF(-)
- Epidemiology: F>M, younger (2-5yo)
- Signs and symptoms: asymmetric, fewer joints, ANA(+)
- Other signs and symptoms: fatigue, malnutrition, delayed growth, anemia, osteopenia
- Differential: Lyme, gonorrhea, viral, SLE, scleroderma, dermatomyositis, spondylarthropathies, malignancy
Enthesitis-related
- Epidemiology: M>F
- Signs and symptoms:
- Inflammation of enthesis (tendon attaches to bone) � heel pain, arthritis
- Sacroiliac joint tenderness, inflammatory spinal pain, HLA-B27, family history, uveitis, pauciarticular or polyarthritis in >8yo M
- If persists: spondyloarthropathies (ankylosing spondylitis, reactive arthritis, IBD-associated arthritis)
Psoriatic
- Signs and symptoms: arthritis, psoriasis, dactylitis, nail pitting (onycholysis), family history of psoriasis
Treatment
- 1st line: NSAIDs (SE: abdominal pain, anorexia)
- Disease-modifying anti-rheumatic drugs (DMARDs): methotrexate plus 1mg folic acid (SE: GI – oral ulcers, decreased appetite, abdominal pain), sulfasalzine, steroids (only short-term for severe manifestations)
- Other – Etanerecpt (TNF-R antagonist), joint replacement surgery, autologous stem cell transplant (last resort)
- PT/OT, splints, screen for uveitis
References
Goldmuntz EA and White PH. Juvenile Idiopathic Arthritis: A Review for the Pediatrician. Pediatrics in Review 2006; 27(4): e24-e32.
Espinosa et al. Juvenile Idiopathic Arthritis. Pediatrics in Review July 2012