B43-41 A Good-natured Journey to Greener Pastures

Introduction Anti-glomerular basement membrane (anti-GMB) disease is an autoimmune vasculitis driven by autoantibodies against collagen found in the kidneys and lungs. An initial environmental, inflammatory, or infectious insult to the renal basement membrane or alveolar capillaries leads to formation of autoantibodies. Then, the complement pathway is activated which perpetuates tissue injury and immunoglobulin deposition. Renal disease is more prevalent and may occur earlier than pulmonary findings. 40-60% of cases have pulmonary involvement, usually with diffuse alveolar hemorrhage, and only 10% occur without renal findings. Renal biopsy allows for diagnosis and prognostication of renal survival. Younger patients are critically ill with pulmonary and renal symptoms unlike older individuals who present solely with glomerulonephritis. Early initiation of immunosuppression and plasmapheresis allows for excellent long-term prognosis. Case Presentation 41-year-old female with PMH of HTN presented to the ED for worsened kidney function. She had experienced 4 weeks of fatigue, weight gain, and hematuria. On presentation, she had a creatinine of 25, a potassium of 6.0 and a sodium of 126. In the emergency department, she had an anion gap metabolic acidosis with a bicarbonate of 8 and was anemic with hemoglobin of 3.4. She received 4 units of blood and was admitted to the ICU.Nephrology started hemodialysis; autoimmune workup was initiated. Hematology concerned for TTP and started plasmapheresis as well as dexamethasone. Patient clinically worsened two days later and became hypoxic. Anti-GBM antibodies returned positive. Chest XR demonstrated worsening bibasilar airspace opacities and increased pulmonary edema, concerning for diffuse alveolar hemorrhage. Patient was emergently intubated and prepped for bronchoscopy. BAL revealed frothy blood-tinged secretions diffusely present throughout the lungs. BAL of the RML was completed, without evidence of infection, showed serial aliquots of hemorrhage. ANCA, ANA, ADAMTS-13 were unremarkable. High dose methylprednisolone initiated for suspicion of anti-GBM disease. She was extubated in 4 days and had a kidney biopsy. Renal biopsy confirmed positive GBM serologies and sclerotic changes consistent with anti-GBM. She was started on cyclophosphamide and daily plasmapheresis with improvement. Discussion This case highlights the need for a broad differential when evaluating acute-onset organ failure. Although stabilization is the initial focus, physicians should strive to elucidate the underlying etiology to prevent disease progression. Early suspicion and assessment of anti-GBM antibodies with prompt initiation of plasmapheresis and immunosuppression leads to increased patient survival. Multidisciplinary teams allow for effective workup and care of this complex disease. This abstract is funded by: None