DIFFUSE ALVEOLAR HEMORRHAGE AS CLINICAL PRESENTATION OF GPA

Abstract

TYPE: Abstract Publication TOPIC: Diffuse Lung Disease PURPOSE: We describe a case of diffuse alveolar hemorrhage (DAH) as a presenting clinical manifestation of ANCA-associated vasculitis (AAV) leading to pulmonary-renal syndrome (PRS), a rare complication with a high mortality rate. METHODS: A 52-yo male presented with a one-week history of malaise and dry cough. Lung examination showed decreased breath sounds. Chest x-ray demonstrated diffuse bilateral opacities. CT showed diffuse alveolar filling. Labs were significant for normocytic anemia 8.1(g/dL), renal failure (Cr 3.14 (mg/dL)) and PaO2 74mm HG on 100% oxygen. Vancomycin, piperacillin/tazobactam, and azithromycin were initiated. Urinalysis showed hematuria and proteinuria. Autoimmune workup revealed CRP 213 mg/L, ESR 56 mm/h, ANCA (+), C-ANCA 1:1280, PR3+ >8. Due to rapidly decreasing hemoglobin and respiratory failure patient was intubated and bronchoscopy confirmed DAH. Patient was treated with pulse steroids for five days, cyclophosphamide, and plasmampharesis. Intermittent hemodialysis initiated for renal failure and anuria. Patient was extubated on day 7 and renal biopsy on day 10 confirmed acute necrotizing glomerulonephritis consistent with AAV. RESULTS: We report a case of GPA with sudden onset of hypoxic respiratory failure progressing to PRS. Severities of presenting symptoms and findings in patients with GPA vary considerably from asymptomatic to acute alveolar hemorrhage with respiratory failure. CONCLUSIONS: Although DAH is a rare manifestation of the disease, occurring only in 5% to 10% of patients, it acts as a precursor of severe vasculitis. CLINICAL IMPLICATIONS: Early detection of GPA is essential since it may lead to rapid clinical deterioration; high of index of suspicion and early management significantly improves morbidity and mortality. DISCLOSURE: No significant relationships. KEYWORDS: Diffuse Alveolar Hemorrhage, ANCA Vasculitis