A 12-year-old girl presents with 4 weeks of daily fever (>39°C spiking once daily with rapid defervescence), salmon-pink evanescent maculopapular rash appearing with fever, arthritis of multiple joints, hepatosplenomegaly, and lymphadenopathy. Serum ferritin is 18,500 ng/mL, RF negative, ANA negative. Which complication is MOST feared in this condition and requires close monitoring?
- A Amyloidosis from chronic inflammation
- B Pulmonary arterial hypertension
- C Macrophage activation syndrome (MAS) characterised by cytokine storm ✓
- D Uveitis leading to blindness
Explanation
Systemic JIA (sJIA) is uniquely associated with macrophage activation syndrome (MAS), a life-threatening hyperferritinaemia syndrome characterised by pathological macrophage activation, cytokine storm (IL-1β, IL-18, IL-6), haemophagocytosis in marrow/liver/spleen, and multi-organ failure. Warning signs: sudden fall in WBC and platelets despite active disease, falling ESR with rising CRP/ferritin, coagulopathy. Ferritin >10,000 ng/mL is a red flag. Uveitis is the feared complication of oligoarticular and RF+ JIA subtypes. Amyloidosis is now rare with modern therapy. Pulmonary hypertension is more associated with scleroderma.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.