Pediatric ANCA-associated vasculitis presented with various clinical findings mimicking IgA Vasculitis and IgG4-related disease: Two cases

Abstract

Granulomatous polyangiitis (GPA) is the most common anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), characterized by necrotizing inflammation of small and medium-sized vessels. It can affect various organs, particularly the lung, kidneys, upper respiratory tract, ears, and skin. Diagnosis of AAV poses significant challenges due to its diverse clinical features. This report presents two interesting cases of GPA: one with rare ocular involvement, who subsequently developed end-stage kidney disease (ESKD), and the other with palpable purpura mimicking immunoglobulin A vasculitis, who relapsed with mastoiditis while in renal remission. Early and effective treatment can improve patient prognosis, highlighting the importance of increasing disease awareness during initial diagnosis and in pediatric AAV patients experiencing relapses.