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A3070 - The Benefit of Plasmapheresis on Alveolar Hemorrhage in Pulmonary-Renal Syndrome: A Case of Granulomatosis with Polyangiitis (GPA)
Author Block: F. Kukhon1, F. Al Shami1, A. Minami1, I. Grillo1, J. Clement2, F. Suhail2, K. Bartolomei1, T. Minami3; 1Brown University / Memorial Hospital of Rhode Island, Pawtucket, RI, United States, 2Memorial Hospital of Rhode Island, Pawtucket, RI, United States, 3Pulmonary, Critical Care and Sleep Medicine, Memorial Hospital of Rhode Island, Pawtucket, RI, United States.
INTRODUCTION: Pulmonary-renal syndrome describes acute glomerulonephritis and pulmonary hemorrhage due to diseases affecting both lungs and kidneys. Examples include antineutrophil-cytoplasmic antibody (ANCA)-positive vasculitis and Goodpasture syndrome. We present a case of pulmonary-renal syndrome due to GPA that improved following treatment with plasmapheresis and cytotoxic therapy.
CASE DESCRIPTION: An 86-year-old woman with history of hypertension was admitted for worsened renal function. The patient reported fatigue and 15-pound weight loss over three months. On presentation, temperature was 37.1 degrees Celsius, blood pressure was 202/81 mm Hg, pulse rate was 98 beats per minute, respiratory rate was 18 breaths per minute, and oxygen saturation was 98% on room air. The remainder of the examination was unremarkable. Laboratory findings were remarkable for creatinine of 5.86 mg/dL and elevated inflammatory markers. On day three of hospitalization, the patient developed hemoptysis and hypoxemia. Computed tomography revealed scattered bilateral lung nodules, cavitation, consolidation, and ground-glass appearance. The patient was transferred to the intensive care unit for acute hypoxemic respiratory failure. She underwent hemodialysis and treatment with high-dose methylprednisolone for possible GPA, as work-up had revealed positive C-ANCA. A kidney biopsy showed crescentic, pauci-immune glomerulonephritis. The biopsy result, along with the pulmonary hemorrhage and the C-ANCA positivity, confirmed the diagnosis of GPA. She continued to require four liters of oxygen and to have hemoptysis. Plasmapheresis was started on day six and her respiratory condition dramatically improved, requiring only one liter of oxygen after seven sessions of plasmapheresis. She was continued on hemodialysis and was also treated with rituximab. Three months after discharge, her kidney function continued to improve and she was taken off dialysis.
DISCUSSION: The role of plasmapheresis in patients with GPA-associated alveolar hemorrhage is still uncertain. All patients with pulmonary hemorrhage should be treated with pulse steroids and either cyclophosphamide or rituximab. Plasma exchange is based on the theoretical benefit of removing ANCA and the known efficacy in patients with pulmonary hemorrhage with anti-glomerular basement membrane (GBM) antibody disease. There are no published randomized trials; however, a retrospective review of 20 patients with alveolar hemorrhage and ANCA-vasculitis showed resolution of alveolar hemorrhage in all patients treated with plasmapheresis. In our case, the patient’s respiratory failure caused by pulmonary hemorrhage improved dramatically with plasmapheresis. This suggests that plasmapheresis could be beneficial in treating alveolar hemorrhage associated with GPA.
CONCLUSION: Plasmapheresis may be beneficial for alveolar hemorrhage in patients with GPA.