Kidney Week

Abstract: SA-PO769

Co-occurrence of Crescentic IgA Nephropathy and Borderline Positive ANCA-Associated Vasculitis: A Perilous Pairing!

Session Information

• ANCA-Associated Vasculitis, Anti-GBM Disease, and Other RPGN
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Sharma Divyadarshini, Divya, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States

Divya Sharma Divyadarshini, MD

• Sharma Priamvada, Gargi, East Carolina University, Greenville, North Carolina, United States

Gargi Sharma Priamvada, MD

• Iskander, Kirolos, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States

Kirolos Iskander, MD

• Ahmad, Zahid Bashir, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States

Zahid Bashir Ahmad, MD

Introduction

The convergence of IgA nephropathy (IgAN) and Antineutrophil cytoplasmic antibody associated vasculitis (AAV) represents a rare but clinically significant condition. This combination presents with severe clinical manifestations, characterized by biopsy-confirmed IgAN, serological positivity for ANCA, rapid progression of renal failure, and pronounced pulmonary involvement. In this case, we underscore the complexities in management and emphasize the grim prognosis associated with IgAN/ANCA-positive vasculitis.

Case Description

46-year-old male with alcoholic cirrhosis presented with epistaxis, anemia, and rapidly increasing creatinine levels (from 6.26 mg/dl to 10 mg/dl within 3 days, baseline unknown) necessitating hemodialysis. Urine-protein creatinine ratio of 2.2 g/g, dysmorphic microhematuria, and borderline positive PR3-ANCA at 1.1 U (normal range: 0.0 - 0.9 U) were noted. Renal biopsy showed minimal interstitial fibrosis, endocapillary hypercellularity, 33% cellular crescents, and granular mesangial staining of IgA and C3 on immunofluorescence. CT imaging detected bilateral lung infiltrates. Bronchoalveolar lavage showed nasal ulcers and extensive diffuse alveolar hemorrhage (DAH). Crescentic IgAN with concurrent AAV was diagnosed. Despite aggressive treatment with high-dose steroids, cyclophosphamide, and plasmapheresis, the patient deteriorated due to persistent DAH complicated by infections, resulting in death.

Discussion

This case of IgAN/ANCA+ highlights the importance of thorough serological assessment and early kidney biopsy in individuals with acute pulmonary-renal syndrome. IgAN infrequently presents as rapidly progressive glomerulonephritis and indicates a poorer prognosis necessitating swift intervention. Per literature review, clinicopathological combination of IgAN/ANCA+ is associated with worse clinical outcomes like DAH and acute renal failure, when compared to IgAN/ANCA- or AAV. Management of such mixed glomerulonephritis is inadequately documented. While high-dose steroids, cyclophosphamide/rituximab, and plasmapheresis are potential treatments, their effectiveness is limited by the risk of fatal infections.