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IgA Vasculitis — SCE Rheumatology MCQ

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EasyVasculitisIgA VasculitisSCE Rheumatology

A 12-year-old boy presents with palpable, non-blanching purpura predominantly affecting his legs and buttocks. He also has colicky abdominal pain and microscopic haematuria. His platelet count is normal. What is the most likely diagnosis?

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Correct answer: CIgA vasculitis (Henoch–Schönlein purpura)

The diagnosis is IgA vasculitis. The characteristic pattern is non-thrombocytopenic purpura with lower-limb predominance accompanied by gastrointestinal and renal involvement. This child has the mandatory purpuric rash together with abdominal pain and microscopic haematuria, satisfying the EULAR/PRINTO/PRES classification pattern. Microscopic polyangiitis can cause glomerulonephritis and purpura but is uncommon in children and does not typically produce this classic dependent rash–abdominal syndrome. Granulomatosis with polyangiitis usually has upper or lower respiratory tract disease. Polyarteritis nodosa is a medium-vessel vasculitis and does not characteristically cause glomerulonephritis. Kawasaki disease presents with prolonged fever and mucocutaneous inflammation, usually in younger children. Serum IgA elevation was removed because it is neither required nor independently diagnostic.

Reference: Ozen S et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura: Ankara 2008, Part II—Final classification criteria. Annals of the Rheumatic Diseases. 2010;69:798–806. https://pubmed.ncbi.nlm.nih.gov/20413568/