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• W4387248002 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 7 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 12:00 pm - 12:45 pm INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a rare pulmonary manifestation of granulomatosis with polyangiitis (GPA). Given its kaleidoscopic variation in presentation, the diagnostic challenge is associated with significant mortality. Early identification of the disease and its targeted treatment is vital in preventing irreversible respiratory and renal complications. CASE PRESENTATION: A 64-year-old male presented to the emergency department with lightheadedness. The patient had no history of dyspnea, hemoptysis, rash, or other systemic symptoms. There was no known medical history. Laboratory investigations revealed normal renal functions on admission but later had rapid deterioration, with serum creatinine reaching up to 4.6 mg/dl. Abdominal ultrasound demonstrated bilateral simple and complex renal cysts with prostatomegaly. Urinalysis revealed proteinuria, hematuria, and granular casts, and urine protein electrophoresis revealed a glomerular pattern. Antiproteinase-3 antibody was elevated. Anti-glomerular basement membrane antibodies were negative. Shortly afterward, the patient had acute onset hypoxic respiratory failure needing mechanical ventilation (MV). Chest X-ray revealed diffuse infiltrates, and complete blood count showed an acute drop in hematocrit (from 22.4% to 17.9%). The patient underwent bronchoscopy with bronchoalveolar lavage which was consistent with the diagnosis of DAH. Based on kidney and lung involvement with positive circulating cytoplasmic-ANCA, the patient met the classification criteria for GPA as per ACR-2022 (American College of Rheumatology) guidelines. He was treated with a pulse dose of steroids and two cycles of rituximab. Subsequent chest imaging revealed marked improvement in infiltrates and stabilization of hematocrit, indicating resolution of DAH. Following a prolonged hospital stay, the renal function improved. He was eventually liberated from MV. DISCUSSION: GPA is a small vessel antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly affects the upper airways, lung parenchyma, and kidneys. Typical lung involvement includes nodules, masses, cavitations, fixed infiltrates, or granulomas. DAH develops in 5-10% of patients and is associated with high mortality. It typically presents with respiratory symptoms ranging from cough, dyspnea, or hemoptysis to acute hypoxic respiratory failure. In patients presenting with AAV, DAH should be suspected in the presence of hypoxia and a drop in hematocrit. As chest imaging is usually nonspecific and hemoptysis is absent in one-third of patients, emergent bronchoscopy is indicated for diagnosis. Bronchoalveolar toileting reveals increasing bloody lavage in serial aliquots, confirming the diagnosis. Treatment is with systemic glucocorticoids and immunomodulators. Rituximab is preferred over cyclophosphamide to control AAV based on recent studies. After disease remission, maintenance therapy for 12-18 months with low-dose glucocorticoids and anti-metabolites such as azathioprine, rituximab, and mycophenolate mofetil remains the standard of care. CONCLUSIONS: The extent of systemic involvement determines the severity of AAV. Involvement of kidneys and pulmonary hemorrhage of any severity differentiates severe, organ-threatening AAV from non-organ-threatening AAV. Our patient demonstrated two major organ involvement at the inception of the disease. Hemoptysis was absent, and a diagnosis of DAH was established by bronchoscopy. This case highlights the significance of sharp clinical vigilance, the use of ancillary testing to diagnose DAH, and the therapeutic benefit of the early institution of a specific therapy. REFERENCE #1: ​​Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315-320. doi:10.1136/annrheumdis-2021-221795 REFERENCE #2: Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest. 2010;137(5):1164-1171. doi:10.1378/CHEST.08-2084 REFERENCE #3: Stone JH. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. Journal fur Mineralstoffwechsel. 2010;17(4):168. doi:10.1056/NEJMOA0909905 DISCLOSURES: No relevant relationships by Tanushree Bhatt No relevant relationships by Ked Fortuzi No relevant relationships by SNEHA KHANAL No relevant relationships by Sindhaghatta Venkatram" @default.
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• W4387248002 date "2023-10-01" @default.
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• W4387248002 title "NEW-ONSET ANTINEUTROPHIL CYTOPLASMIC ANTIBODY VASCULITIS UNMASKED AFTER AN EPISODE OF LIFE-THREATENING DIFFUSE ALVEOLAR HEMORRHAGE" @default.
• W4387248002 doi "https://doi.org/10.1016/j.chest.2023.07.3609" @default.
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