Abstract Introduction Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a necrotizing vasculitis that affects small vessels and varies in presentation and severity. It commonly involves the kidneys and respiratory tract, manifesting as necrotizing glomerulonephritis and/or pulmonary capillaritis. Among its complications, diffuse alveolar hemorrhage (DAH) is one of the most life-threatening. When both the lungs and kidneys are affected, patients often experience rapid clinical deterioration. Early recognition and initiation of immunosuppressive therapy are essential to improve survival outcomes. Current guidelines recommend glucocorticoids in combination with either rituximab or cyclophosphamide for induction. The role of plasma exchange remains controversial and is not routinely recommended, even in cases of pulmonary hemorrhage. We present a case of severe AAV in which prompt diagnosis and treatment were key to recovery. Case Presentation A 79-year-old female with hypertension and pulmonary hypertension presented with progressive unintentional weight loss, malaise, and weakness over several weeks. On the day of admission, she developed acute dyspnea and hemoptysis. Initial laboratory evaluation revealed acute kidney injury (creatinine 2.7 mg/dL; previously normal) and severe anemia (hemoglobin 5.4 g/dL). Urinalysis revealed hematuria and proteinuria. CT chest demonstrated diffuse bilateral ground-glass opacities. She subsequently developed respiratory failure requiring intubation. The combination of pulmonary and renal findings raised strong suspicion for pulmonary-renal syndrome. She was started on IV methylprednisolone. Due to the severity of her presentation and concern for alveolar hemorrhage, plasmapheresis was initiated while awaiting further diagnostic workup, including a kidney biopsy. Because of a delay in obtaining kidney biopsy and high suspicion for vasculitis, empiric cyclophosphamide was started. Bronchoscopy with bronchoalveolar lavage (BAL) confirmed DAH. Serologic testing demonstrated p-ANCA/anti-MPO positivity (128 AU/mL, ref 0–19) and c-ANCA/anti-PR3 mildly elevated (30 AU/mL, ref 0–19). She completed seven sessions of plasmapheresis, with repeat BAL showing resolution of hemorrhage. Kidney biopsy ultimately revealed focal necrotizing and crescentic glomerulonephritis, consistent with AAV. The patient was successfully extubated after one week and continued to improve clinically. Two weeks after induction therapy, rituximab was initiated for maintenance immunosuppression. Discussion AAV presenting with both glomerulonephritis and DAH can be a silent yet life-threatening condition. Early clinical recognition and prompt initiation of aggressive immunosuppressive therapy are essential to improve outcomes. While plasma exchange is not standard of care, it was a critical component of this patient’s recovery. This case highlights the importance of early diagnosis, rapid immunosuppressive initiation, and multidisciplinary management to improve outcomes in severe AAV. This abstract is funded by: None
Jammoa et al. (Fri,) studied this question.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: