Abstract Introduction Systemic lupus erythematosus (SLE) is a complex autoimmune disease with many potential complications. Diffuse alveolar hemorrhage (DAH) is rare and one of the most life-threatening, estimated to affect 0.5-5.7% of adults with SLE with an average mortality of 50%. The main treatment is high dose glucocorticoids, often with cyclophosphamide, however, other therapies including rituximab, therapeutic plasma exchange, and IVIG have also been tried. In patients with persistent hypoxemia despite intubation, extracorporeal membrane oxygenation (ECMO) has occasionally been reported with mixed success, especially given the increased bleeding risk associated with systemic anticoagulation. In our case, we present a patient, non-adherent to immunosuppressant therapy, who survived SLE-associated DAH by using ECMO with systemic anticoagulation. Description A 46 year-old female with type B insulin resistance, SLE complicated by pericardial effusion and nephritis, numerous deep vein thromboses and pulmonary emboli, who was nonadherent with immunosuppressants and anticoagulation, was admitted for pulmonary edema seen on outside chest x-ray. Patient was initially tachycardic but not hypoxic, with crackles at bilateral lung bases. CT PE showed chronic pulmonary emboli and persistent moderate pericardial effusion. Less than 12 hours later, she developed acute hypoxic respiratory failure with worsening anemia. Repeat CT at that time revealed diffuse ground-glass opacities suspicious for hemorrhage. Despite mechanical ventilation on maximal settings and inhaled nitrous oxide, P:F was 70 indicating severe acute respiratory distress syndrome. She was cannulated for VV ECMO and received systemic anticoagulation, with bronchoscopy revealing DAH. She received methylprednisolone 500mg for 3 days, was decannulated on day 4, extubated on day 5, and continued to improve thereafter. Discussion DAH is often a catastrophic complication of SLE necessitating aggressive treatment, and when hypoxemia persists despite maximal ventilator support, fatality is often the result. While ECMO is still an uncommon treatment modality for SLE-DAH, our case shows that ECMO with systemic anticoagulation can be a successful, life-saving intervention for these patients. This abstract is funded by: N/A
Janczewski et al. (Fri,) studied this question.
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