SESSION TITLE: Critical Care Case Report Posters II
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is recognized by the constellation of hemoptysis, anemia, diffuse radiographic pulmonary infiltrates, and hypoxemic respiratory failure. Common causes include Granulomatosis with polyangiitis, Good-pasture syndrome, and collagen vascular diseases.
CASE PRESENTATION: A 44 year old Ethiopian female with past medical history of hepatitis C and primary biliary cirrhosis underwent upper endoscopy for chronic anemia. Following the procedure, patient became hypoxic. Chest X-ray revealed bilateral alveolar infiltrates and her respiratory status continued to decline requiring intubation. Further history revealed ongoing hemoptysis for last 6 months with progressive shortness of breath. Initial laboratory work-up showed acute kidney injury and dysmorphic red blood cells in the urine suggestive of nephritic process. On ICU day 1, diagnostic bronchoscopy with bronchoalveolar lavage (BAL) revealed diffuse alveolar hemorrhage. Pulse dose steroids and plasma exchange were started immediately. Blood work was negative for ANA, ANCA, anti GBM antibody and HIV but low complement levels and elevated rheumatoid factor (2170). Hepatitis C RNA was elevated at 4 million IU/ml. Immunofixation studies were eventually positive for cryoglobulin type-2. Anti-viral therapy (entecavir) for Hepatitis C and rituximab were also started with excellent response and resolution of respiratory failure. Kidney function continued to decline and necessitated dialysis. On hospital day 25, she developed refractory septic shock and expired.
DISCUSSION: Cryoglobulinemic vasculitis is a systemic vasculitis associated with serum positive cryoglobulins which are immune complexes composed of rheumatoid factor (RF), usually linked to non- malignant B-cell lymphoproliferation and often triggered by chronic hepatitis C virus (HCV) infection. Most patients have renal and gastrointestinal involvement. Highest mortality rate is observed with pulmonary involvement (though only 7% patients), primarily due to septic shock or vasculitic process itself.
CONCLUSIONS: DAH due to HCV-related cryoglobulinemia is associated with a high risk of mortality and should be treated aggressively with pulse steroids, combined with plasma exchanges and/or rituximab, followed by antiviral therapy.
Reference #1: Lara et al. Diffuse Alveolar Hemorrhage. CHEST 2010
Reference #2: De Vita et al Preliminary classification criteria for the cryoglobulinemic vasculitis. Ann Rheum Dis 2011
Reference #3: Retamozo et al. Life-Threatening Cryoglobulinemic Patients With Hepatitis C. Medicine 2013
DISCLOSURE: The following authors have nothing to disclose: Puneet Agarwal, Michael Warner, Mark Abi Nader, Cristina Reichner, Tunay Kuru
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