A 6-year-old girl has fever >39°C for 3 weeks, arthritis of multiple joints, salmon-pink evanescent rash that appears with fever and disappears when afebrile, hepatosplenomegaly, lymphadenopathy, and serositis (pericarditis). RF and ANA are negative. Ferritin is 18,000 ng/mL. What is the diagnosis?
- A Systemic lupus erythematosus (SLE)
- B Kawasaki disease
- C Acute rheumatic fever
- D Systemic juvenile idiopathic arthritis (sJIA) / Still's disease ✓
Explanation
Systemic JIA (Still's disease) is characterised by quotidian fever (>39°C, once or twice daily, returns to normal or below) for ≥2 weeks, at least one joint involved, AND one of: evanescent salmon-pink rash (pathognomonic, appears during fever spikes), lymphadenopathy, hepatosplenomegaly, or serositis. Very high ferritin (often >10,000 ng/mL) is characteristic and reflects macrophage activation. RF and ANA are typically negative. The dreaded complication is macrophage activation syndrome (MAS). SLE has ANA+/dsDNA+, butterfly rash, renal involvement. Kawasaki occurs in younger children with conjunctivitis and mucous membrane changes. ARF has streptococcal evidence and Jones criteria.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.