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A3052 - Diffuse Alveolar Hemorrhage Necessitating Extracorporeal Membrane Oxygenation Rescue Therapy
Author Block: E. Beck1, L. M. Keenan2; 1University of Utah, Salt Lake City, UT, United States, 2University of Utah and George E. Whalen Department of Veterans Affairs Medical Center, Salt Lake City, UT, United States.
Introduction Pulmonary hemorrhage is a rare and life-threatening complication of systemic lupus erythematosus (SLE). Diffuse alveolar hemorrhage (DAH) in SLE is thought to be secondary to capillaritis and bland hemorrhage and usually responds to high-dose corticosteroids with or without other immunosuppressive agents. Here, we present a case of severe, refractory SLE-related DAH requiring rescue extracorporeal membrane oxygenation (ECMO).
Case Presentation A 52 year-old woman with SLE complicated by nephritis was admitted to the ICU for hypoxemic respiratory failure related to hemoptysis necessitating endotracheal intubation and mechanical ventilation. Bronchoscopic findings confirmed DAH. High-dose steroids were added to her regimen of mycophenolate mofetil and hydroxychloroquine. She continued to have progressive hemoptysis with impaired oxygenation and a second bronchoscopy was performed with instillation of topical thrombin and epinephrine into the LUL where active bleeding was previously seen. Hemostasis was confirmed visually.
Preparations were made for instituting plasmapheresis given rapid progression of her disease activity in the setting of optimal medical management. Placement of a hemodialysis catheter was complicated by ventricular arrhythmia with cardiac arrest. She underwent CPR with restoration of cardiac function but subsequent hemodynamic collapse and copious bloody secretions with progressive refractory hypoxemia despite FiO2 1.0 and PEEP 15 cm H2O. The ECMO team was mobilized and VA ECMO was initiated for hemodynamic and respiratory support.
While undergoing ECMO, she received cyclophosphamide, plasmapheresis and transfusion support due to oozing from catheter sites while on systemic anticoagulation. Her cardiopulmonary status improved over the following 6 days; ECMO lines were removed and she was extubated on hospital day 7. Her hospital course was prolonged and complicated by progressive renal failure necessitating hemodialysis, GI bleeding, and recurrent episodes of respiratory failure related to recurrent DAH and opportunistic infections. She was discharged to a long term acute facility after 2.5 months.
Discussion Pulmonary hemorrhage occurs in 0.5 - 5.4% of lupus patients with a >50% mortality; higher for those requiring mechanical ventilation. First-line treatment involves high doses of corticosteroids followed by a second immunosuppressive agent. Severe, refractory cases may require mechanical ventilation and consideration of plasma exchange or other toxic intensive therapies such as cyclophosphamide or rituximab. Although controversial, ECMO is indicated in potentially reversible severe acute cardiac or respiratory failure unresponsive to conventional therapy. Uncontrolled active bleeding is a relative contraindication due to need for systemic anticoagulation. When instituted in the setting of active hemorrhage, careful attention to and adjustments in anticoagulation may be necessary.