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Abstract: SA-PO0918

More than Skin Deep: Successful Management of Rare IgA Vasculitis with Kidney Involvement

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Sharma Divyadarshini, Divya, The University of Oklahoma Health Sciences College of Medicine, Oklahoma City, Oklahoma, United States
  • Tjauw, Maxmillian J, The University of Oklahoma Health Sciences College of Medicine, Oklahoma City, Oklahoma, United States
  • Sharma Priamvada, Gargi, East Carolina University, Greenville, North Carolina, United States
  • Ahmad, Zahid Bashir, The University of Oklahoma Health Sciences College of Medicine, Oklahoma City, Oklahoma, United States
Introduction

IgA vasculitis (IgAV), formerly Henoch-Schönlein purpura, is a small-vessel vasculitis marked by IgA-dominant immune complex deposition. While common in children, adult-onset IgAV has an incidence of 0.8–2 cases per 100,000 adults annually in the United States. Adults often experience more severe manifestations, particularly renal involvement, and have worse prognosis if diagnosis is delayed. This case highlights the benefits of early kidney biopsy and prompt initiation of treatment, leading to significant clinical and renal improvement in an adult-onset IgAV presentation.

Case Description

A 30-year-old female presented with palpable purpura on her legs, fatigue, arthralgias, and recent diarrhea with hematochezia . Hemoglobin, platelets and coagulation studies were normal. UA urinalysis showed microscopic hematuria with abundant acanthocytes under microscopy; Sr. Cr. rose from 0.9 to 1.4 mg/dL. 24-hour urine protein was 2.4 g/day. Skin biopsy revealed leukocytoclastic vasculitis, and kidney biopsy showed IgA-dominant mesangial deposits with hypercellularity and crescents in 33% of glomeruli. Immunofluorescence confirmed granular mesangial IgA deposition. Diagnosed with IgAV with IgA dominant glomerulonephritis, she was started on prednisone, mycophenolate mofetil (MMF), and lisinopril. She achieved complete remission within 16 weeks of therapy.

Discussion

This case highlights the need to understand the systemic manifestations of IgAV for rapid diagnosis with skin or kidney biopsy and early, aggressive treatment. RAAS blockade, corticosteroids and cyclophosphamide or MMF are mainstay of treatment, but newer drugs like sparsentan and iptacopan, specifically targeting the immune system, have shown promise.

From left to right: Gross specimen minimal interstitial fibrosis, IF granular mesangial IgA deposition, LM IgA-dominant mesangial deposits with mesangial hypercellularity and crescent.

Digital Object Identifier (DOI)