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• W4304146105 abstract "SESSION TITLE: Critical Systemic Disease Case Report PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/19/2022 12:45 pm - 01:45 pmINTRODUCTION: Microscopic polyangiitis is a small vessel vasculitis that results from an autoimmune activation of neutrophils driven by anti-neutrophil cytoplasmic antibodies (ANCA). The activated neutrophils release inflammatory cytokines, reactive oxygen species, and lytic enzymes that result in necrotizing granulomatous inflammation leading to damage to the small and medium blood vessels, most commonly in the lungs, kidneys, and nervous system. First-line therapy for ANCA vasculitis is corticosteroids, however when there is organ-threatening damage, treatment is escalated to cyclophosphamide or rituximab. Rituximab is the treatment of choice for relapsing disease. In patients with acute renal failure or pulmonary hemorrhage, plasmapheresis can be considered, however, there has not been strong evidence to suggest that plasmapheresis reduces all-cause mortality [1,2].CASE PRESENTATION: A 66-year-old African American male with recent diagnosis of p-ANCA glomerulonephritis status-post Rituximab induction therapy presented to the emergency department with insidious onset shortness of breath. He denied cough, chills, fever, chills or hemoptysis. On arrival he was afebrile, tachypneic, and requiring high-flow nasal cannula oxygen. On physical examination he was ill appearing, and crackles were auscultated diffusely. He remained hypoxic requiring rapid escalation to non-invasive positive pressure ventilation at 100% Fi02. Chest x-ray showed ground glass opacities throughout, which was further characterized on high resolution CT scan as extensive bilateral reticular pulmonary parenchymal opacities (figure 1). Broad spectrum antibiotics as well as high dose steroids were initiated. His respiratory status continued to decline requiring transfer to the intensive care unit and eventual intubation. His steroid regimen was increased to pulse dose - 1g daily for 3 days. Bronchoalveolar lavage was consistent with diffuse alveolar hemorrhage and plasmapheresis was initiated. Unfortunately, he developed worsening septic shock and multi-system organ failure. His family opted to pursue comfort measures only and he succumbed to illness on hospital day five.DISCUSSION: Pulmonary involvement is a common complication of MPA affecting 25-55% of patients [2]. However, pulmonary manifestations cross a spectrum of disease from simple infiltrates to diffuse alveolar hemorrhage (DAH). DAH portends a poor prognosis and higher rate of relapse. Rituximab has superior rates of remission compared to cyclophosphamide or steroids alone. In patients treated with rituximab 94% reached appropriate B-cell depletion [3], therefore in relapse cases it is suggested to reassess for B-cell depletion.CONCLUSIONS: In this case, our patient had adequate B-cell depletion but progressed to pulmonary involvement with rapid deterioration. Hemoptysis is not always a presenting symptom in some patients with diffuse alveolar hemorrhage.Reference #1: Yates M, Watts RA, Bajema IM, et alEULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis Annals of the Rheumatic Diseases 2016;75:1583-1594.Reference #2: Collins CE, Quismorio FP Jr. Pulmonary involvement in microscopic polyangiitis. Curr Opin Pulm Med. 2005;11(5):447-451. doi:10.1097/01.mcp.0000170520.63874.fbReference #3: Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-232. doi:10.1056/NEJMoa0909905DISCLOSURES: No relevant relationships by Kyle FrattaNo relevant relationships by Navitha RameshNo relevant relationships by Michaela Sangillo SESSION TITLE: Critical Systemic Disease Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Microscopic polyangiitis is a small vessel vasculitis that results from an autoimmune activation of neutrophils driven by anti-neutrophil cytoplasmic antibodies (ANCA). The activated neutrophils release inflammatory cytokines, reactive oxygen species, and lytic enzymes that result in necrotizing granulomatous inflammation leading to damage to the small and medium blood vessels, most commonly in the lungs, kidneys, and nervous system. First-line therapy for ANCA vasculitis is corticosteroids, however when there is organ-threatening damage, treatment is escalated to cyclophosphamide or rituximab. Rituximab is the treatment of choice for relapsing disease. In patients with acute renal failure or pulmonary hemorrhage, plasmapheresis can be considered, however, there has not been strong evidence to suggest that plasmapheresis reduces all-cause mortality [1,2]. CASE PRESENTATION: A 66-year-old African American male with recent diagnosis of p-ANCA glomerulonephritis status-post Rituximab induction therapy presented to the emergency department with insidious onset shortness of breath. He denied cough, chills, fever, chills or hemoptysis. On arrival he was afebrile, tachypneic, and requiring high-flow nasal cannula oxygen. On physical examination he was ill appearing, and crackles were auscultated diffusely. He remained hypoxic requiring rapid escalation to non-invasive positive pressure ventilation at 100% Fi02. Chest x-ray showed ground glass opacities throughout, which was further characterized on high resolution CT scan as extensive bilateral reticular pulmonary parenchymal opacities (figure 1). Broad spectrum antibiotics as well as high dose steroids were initiated. His respiratory status continued to decline requiring transfer to the intensive care unit and eventual intubation. His steroid regimen was increased to pulse dose - 1g daily for 3 days. Bronchoalveolar lavage was consistent with diffuse alveolar hemorrhage and plasmapheresis was initiated. Unfortunately, he developed worsening septic shock and multi-system organ failure. His family opted to pursue comfort measures only and he succumbed to illness on hospital day five. DISCUSSION: Pulmonary involvement is a common complication of MPA affecting 25-55% of patients [2]. However, pulmonary manifestations cross a spectrum of disease from simple infiltrates to diffuse alveolar hemorrhage (DAH). DAH portends a poor prognosis and higher rate of relapse. Rituximab has superior rates of remission compared to cyclophosphamide or steroids alone. In patients treated with rituximab 94% reached appropriate B-cell depletion [3], therefore in relapse cases it is suggested to reassess for B-cell depletion. CONCLUSIONS: In this case, our patient had adequate B-cell depletion but progressed to pulmonary involvement with rapid deterioration. Hemoptysis is not always a presenting symptom in some patients with diffuse alveolar hemorrhage. Reference #1: Yates M, Watts RA, Bajema IM, et alEULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis Annals of the Rheumatic Diseases 2016;75:1583-1594. Reference #2: Collins CE, Quismorio FP Jr. Pulmonary involvement in microscopic polyangiitis. Curr Opin Pulm Med. 2005;11(5):447-451. doi:10.1097/01.mcp.0000170520.63874.fb Reference #3: Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-232. doi:10.1056/NEJMoa0909905 DISCLOSURES: No relevant relationships by Kyle Fratta No relevant relationships by Navitha Ramesh No relevant relationships by Michaela Sangillo" @default.
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• W4304146105 title "RAPIDLY PROGRESSIVE MICROSCOPIC POLYANGIITIS WITH DIFFUSE ALVEOLAR HEMORRHAGE" @default.
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