IgA vasculitis (IgAV) and IgA nephropathy (IgAN) are increasingly recognized as part of a disease spectrum sharing common pathogenic mechanisms involving galactose-deficient IgA1. While IgAN represents kidney-limited disease, IgAV is the systemic manifestation. The development of systemic IgA vasculitis in patients with established end-stage renal disease (ESRD) secondary to IgA nephropathy is rarely reported and presents unique diagnostic challenges. We present a case of a 51-year-old woman with ESRD secondary to IgA nephropathy on maintenance hemodialysis who presented with acute anemia, abdominal pain, arthralgias, and gastrointestinal bleeding. On hospital day three, she developed palpable purpura on the bilateral lower extremities. Skin biopsy demonstrated leukocytoclastic vasculitis with perivascular IgA, IgM, and C3 deposition on direct immunofluorescence, confirming IgA vasculitis. Treatment with high-dose systemic corticosteroids resulted in the resolution of cutaneous and systemic manifestations. This case highlights the diagnostic complexity of adult IgA vasculitis in ESRD patients with underlying IgA nephropathy, where symptoms may mimic common dialysis-related complications. Recognition of the shared pathophysiology between IgAN and IgAV is essential for appropriate diagnosis and management.
Sharma et al. (Fri,) studied this question.