Focal Alveolar Hemorrhage: A Unique Presentation of ANCA-Associated Vasculitis

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease SESSION TYPE: Case Report Slide PRESENTED ON: Saturday, April 16, 2016 at 09:45 AM - 11:15 AM INTRODUCTION: Pulmonary renal syndromes are rare clinical entities that can present with diffuse alveolar hemorrhage (DAH) and glomerulonephritis. They are often a result of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV). Typically, alveolar involvement in pulmonary renal syndromes is multi-lobar and bilateral. We describe a case of crescentic glomerulonephritis presenting with hemoptysis and focal alveolar involvement limited to the left lung. CASE PRESENTATION: A 58-year-old woman presented to the hospital reporting a one week history of 2-4 teaspoons of hemoptysis daily with left-sided pleuritic pain. On further assessment, she was found to have acute kidney injury with microhematuria and proteinuria. P-ANCA and myeloperoxidase (MPO) antibody were positive. Acid-fast studies and cultures of blood, urine, and sputum were negative. Bronchoscopy was done showing hemorrhage localized to the left upper lobe. Renal biopsy showed crescentic glomerulonephritis without evidence of granulomas consistent with microscopic polyangiitis. DISCUSSION: AAVs are a small group of vasculitides which include granulomatosis with polyangiitis, microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis, and pauci-immune vasculitis. ANCAs trigger a self-perpetuating inflammatory state when they bind to antigens in the cytoplasmic granules of neutrophils. In the lungs, this inflammation results in diffuse alveolar hemorrhage. In our patient, the constellation of p-ANCA and MPO antibodies, hemoptysis, and crescentic glomerulonephritis without granulomas is highly indicative of MPA. This unique presentation of focal hemorrhage demonstrates that although bilateral disease is classic, AAV should not be excluded in cases of unilateral involvement. Furthermore, repeat imaging over the next 48 hours demonstrated progression to bilateral disease, suggesting DAH may begin as focal disease that generalizes in time. Our patient received high dose steroids, plasma exchange, and rituximab. She was discharged home with improving renal function and without further episodes of hemoptysis. CONCLUSIONS: Although AAV with pulmonary hemorrhage usually presents as a diffuse process, this case demonstrates that it may initially present as a focal area of hemorrhage. These findings underscore the importance of maintaining a high degree of clinical suspicion in patients who do not present classically so that aggressive treatment can be initiated. Reference #1: Gómez-Puerta, J. A., Hernández-Rodríguez, J., López-Soto, A., & Bosch, X. (2009). Antineutrophil cytoplasmic antibody-associated vasculitides and respiratory disease. CHEST Journal, 136(4), 1101-1111. Reference #2: Lally, L., & Spiera, R. F. (2015). Pulmonary Vasculitis. Rheumatic Disease Clinics of North America, 41(2), 315-331. Reference #3: Lichtenberger, J. P., Digumarthy, S. R., Abbott, G. F., Shepard, J. A. O., & Sharma, A. (2014). Diffuse pulmonary hemorrhage: clues to the diagnosis. Current problems in diagnostic radiology, 43(3), 128-139. DISCLOSURE: The following authors have nothing to disclose: Kosal Seng, Timothy Vossler, Michael Kendall, Santhi Iyer Kumar No Product/Research Disclosure Information