Abstract: PUB140
Double Trouble: Life-Threatening ANCA and Anti-GBM Pulmonary Renal Syndrome
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Patel, Jinal, Rush University Medical Center, Chicago, Illinois, United States
- Cimbaluk, David J., Rush University Medical Center, Chicago, Illinois, United States
- Rodby, Roger A., Rush University Medical Center, Chicago, Illinois, United States
Introduction
Anti-glomerular basement membrane (GBM) disease is characterized by an antibody against type IV collagen in the glomeruli and alveoli. This presents as rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage (DAH). Ten to thirty percent of patients with anti-GBM glomerulonephritis (GN) will also test positive for ANCA.
Case Description
A 66-year-old man with ANCA positive interstitial lung disease (ILD) on no therapy presented with an acute kidney injury after influenza A infection. A renal biopsy demonstrated crescentic GN with linear IgG in all glomeruli (Fig. 1a & b). Serum anti-GBM and myeloperoxidase antibodies came back positive. He was started on high dose steroids, plasmapheresis (PLEX) and dialysis. His chest x-ray (CXR) and bronchoalveolar lavage (BAL) were normal, but on day 12 he developed hypoxic respiratory failure requiring intubation. A repeat CXR (Fig. 1c) showed patchy opacities, and BAL showed DAH with a drop in hemoglobin to 6.4 g/dL. Cyclophosphamide was started but he continued to have alveolar hemorrhage, so rituximab was administered. He required 8 blood transfusions throughout his hospital course (Fig 2). His pulmonary hemorrhage eventually abated but renal function did not recover.
Discussion
Anti-GBM disease is often preceded by lung injury, exposing antigens for an autoimmune response, which he was at risk for with ANCA positive ILD and an influenza A infection. Both ANCA vasculitis and anti-GBM disease can cause crescentic GN and pulmonary hemorrhage as classic pulmonary renal syndromes. His DAH was very delayed in the hospital course. While his lungs recovered, his kidney function did not as is common with anti-GBM GN. He is currently being evaluated for a renal transplant. Anti-GBM disease rarely recurs in a transplant, while ANCA vasculitis may.