Abstract Cocaine use is known to be linked to the development of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis, often attributed to levamisole-adulterated formulations. Serology classically exhibits dual positivity for myeloperoxidase (MPO) and proteinase-3 (PR3) antibodies in addition to positive ANCA. While most cases present with fever, arthralgias, and limited cutaneous or sinonasal involvement, severe systemic manifestations are rarer. We present a case of a 48 year-old female with a past medical history of polysubstance use disorder and treated tuberculosis who presented to a hospital on the U.S.-Mexico border with severe pulmonary-renal syndrome requiring massive blood transfusions, initiation of hemodialysis, and placement on extracorporeal membrane oxygenation (ECMO) therapy. For the month prior to presentation the patient had used cocaine heavily following the death of her brother. Three days prior to presentation she developed progressive fatigue, weakness, and shortness of breath for which she decided to present. On arrival the patient was saturating well on room air but was found to have a Hgb of 7.0 g/dL, an AKI with Cr 1.7 mg/dL, and nonspecific bilateral scattered infiltrates on chest-x-ray. She was admitted for RBC transfusion and treatment of presumed pneumonia. However, on hospital Day 2 the patient developed acute hypoxemic respiratory failure secondary to severe ARDS requiring sedation, paralysis, intubation and transfer to the ICU. In the ICU the patient developed large volume bloody secretions suggestive of diffuse alveolar hemorrhage as well as deteriorating renal function requiring the initiation of hemodialysis. Pulmonary-renal syndrome secondary to levamisole-adulterated cocaine was suspected, which was confirmed with ANCA serology positive for p-ANCA 1:160. Despite immunosuppressive glucocorticoid therapy and aggressive intervention, the patient’s cardiopulmonary status declined necessitating transition to ECMO salvage therapy. On ECMO the patient persisted with significant alveolar bleeding requiring massive transfusions. However, after 2 weeks of therapy the patient was able to be weaned off ECMO, downgraded to the hospital floor, and ultimately discharged home. This case highlights the need for high clinical suspicion of ANCA-associated vasculitis in patients with a strong history of cocaine use presenting with anemia, acute kidney injury, and dyspnea. Furthermore, it demonstrates that even initially mild presentations can quickly progress to severe systemic pulmonary and renal manifestations. Prompt serologic testing and aggressive immunosuppressive therapy are key for diagnosis and management to avoid clinical deterioration. However, should it be needed ECMO therapy can serve as a life-saving bridge to recovery in patients with fulminant cocaine-induced ANCA vasculitis. This abstract is funded by: None
Haines et al. (Fri,) studied this question.