LAUSR

Introduction: Diffuse alveolar hemorrhage (DAH) is a syndrome characterized by bleeding into alveolar spaces; it’s often a lifethreatening complication of various conditions. Its association with autoimmune hepatobiliary diseases is not as common as other systemic autoimmune diseases. We describe a patient with primary sclerosis cholangitis (PSC) who developed DAH due to pulmonary vasculitis. This case is significant given very few DAH cases have been reported in a PSC setting. Description: A 37-year-old male with a history of PSC who subsequently developed liver cirrhosis presented to the ED with 2 days of hemoptysis and dyspnea. On examination, he was normotensive, tachycardic, afebrile, and saturating 100% at room air. Pertinent labs: Hg. 6.7g/dL, platelet count 129,000/mL, INR 1.7, PTT 19.7, AST 188 IU/L, ALT 98 IU/L, ALK 1091 IU/L and T. bili 21mg/dL. CT chest was pertinent for crazy paving pattern of the lungs with biapical predominance. He was transfused and started on antibiotics. Bronchoscopy yielded a hemorrhagic return, but no organism was cultured. DAH was diagnosed. Serologies were not contributory but for elevated sedimentation rate and C-reactive protein. He was started on IV high-dose steroids, but hemoptysis continued. Repeated CT chest showed worsening of DAH. He was lethargic and developed progressive hypoxic respiratory failure requiring mechanical ventilation. He was started on plasma exchange for 5 days with steroids. Hemoptysis was resolved, and Hg stabilized and improved. He fully recovered and was discharged. Discussion: DAH is a rare, yet serious syndrome clinically characterized by dyspnea, hemoptysis, anemia, and diffuse bilateral alveolar infiltrates on chest radiograph. Diagnosis is made with a broncoalveolar lavage showing hemorrhagic return and hemosiderin-laden macrophages on cytology. The causes include infections, coagulation disorder, certain drugs and systemic autoimmune disease such as anti-glomerular basement disease and systemic lupus erythematosus. The treatment usually consists in treating the underlying cause often with systemic glucocorticoids and immunosuppressive therapy, such as cyclophosphamide, rituximab, or plasmapheresis. Early recognition is essential because the initiation of a supportive measure and immunotherapy is important for survival.