Abstract: PUB255
ANCA-Associated Vasculitis Initially Misdiagnosed as a Case of Decompensated Heart Failure and Pneumonia
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Kanwal, Fnu, Icahn School of Medicine at Mount Sinai/Elmhurst Hospital Center, Elmhurst, New York, United States
- Stern, Aaron S., Elmhurst Hospital Center, Elmhurst, New York, United States
- Maldonado, Dawn, Elmhurst Hospital Center, Elmhurst, New York, United States
- Stillman, Isaac Ely, Mount Sinai Hospital Pathology and Laboratory Medicine, Manhattan, New York, United States
Introduction
ANCA associated vasculitis is a rare cause of rapidly progressive glomerulonephritis and pulmonary involvement, often presenting as a diagnostic challenge. Early recognition and treatment are crucial to prevent irreversible organ damage.
Case Description
A 76-year female with history of preserved heart failure, and hypertension presented with acute hypoxic respiratory failure secondary to decompensated heart failure with preserved ejection fraction and pneumonia. Admission labs revealed acute kidney injury with a Cr 2.35 mg/dl (baseline Cr 0.7-1.0), urine protein creatinine ratio 2.5 mg/dL and Hemoglobin 9.1g/dL. Urinalysis showed white blood cells, protein, and red blood cells. Chest x-ray showed multifocal interstitial opacification. CT Chest revealed extensive bilateral ground glass opacities without focal consolidation. Autoimmune workup was positive for ANA 1:32, Anti-dsDNA 63, positive for P -ANCA and anti-MPO titer (1:1280), MPO Ab >170 with negative C-ANCA. All other work up was negative. Renal biopsy showed pauci-immune crescentic glomerulonephritis with 84% non- obsolete glomeruli with mild to moderate IFTA. The patient was treated with pulse-dose steroids, rituximab and cyclophosphamide. Hemodialysis was initiated due to worsening kidney function. Her hospital course was complicated by persistent anemia and thrombocytopenia attributed to immunosuppression, and bone marrow biopsy was unremarkable. She required multiple transfusions and was started on romiplostim. She ultimately went into renal recovery and was able to stop dialysis after 10 weeks. She is currently on a steroid taper with excellent urine output and stable renal function.
Discussion
This case highlights the importance of early recognition of ANCA-associated vasculitis. Prompt immunosuppressive therapy and supportive care, including temporary dialysis can lead to renal recovery even in elderly patients with severe disease and multiple complications.
Sclerotic tuft surrounded by a cellular crescent (Elastic Trichrome, Original magnification 40X).