The kitchen sink: Cardiac arrest requiring veno-venous extracorporeal membrane oxygenation, plasma exchange, steroids, and rituximab in a case of granulomatosis with polyangiitis

Background: Small-vessel, anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitis refers to a group of rare diseases defined by a necrotizing, inflammatory process involving the renal and respiratory systems with absence of immune deposits. Diagnosis occurs during adulthood and can present either slowly over a few months or explosively in a few days. The process of developing these disease-specific antibodies is still an unknown. Therefore, management of severe vasculitis with life-threatening manifestations and end-organ damage including pulmonary-renal syndromes remains a highly debated topic. The recent large multicenter trial, PEXIVAS (New England Journal of Medicine, 2020), suggesting no renal or mortality benefit from plasma exchange has elicited pause among providers to reconsider the gold standard of therapy. We describe a success story that used almost every available treatment modality. Case Presentation: 58-year-old female with history of sinusitis and arthritis presented to an outside hospital with multiple nonspecific complaints including dyspnea, cough, and flu-like symptoms. The patient quickly decompensated requiring intubation complicated by need for emergent cricothyroidotomy given extensive amounts of blood in her upper airway. She had subsequent shock and cardiopulmonary arrest. She received multiple large-bore chest tubes due to suspected respiratory arrest from bilateral tension hemopneumothoraces. She received massive transfusion and was cannulated for veno-venous extracorporeal membrane oxygenation (VV-ECMO) due to refractory hypoxia. Diffuse alveolar hemorrhage was seen on bronchoscopy and she developed progressive glomerulonephritis requiring continuous renal replacement therapy. She was diagnosed with c-ANCA positive vasculitis on hospital day 3. She received plasmapheresis, high dose steroids, and rituximab. The patient recovered and was decannulated from VV-ECMO and tracheostomy on hospital day 8 and 28, respectively. She was discharged home and was no longer hypoxic or on dialysis at post-discharge day 25 Conclusion: Our patient is an example of the severe and explosive consequences of ANCA-associated vasculitis with mobilization of nearly every applicable therapy in critical care which ultimately saved her life. As the medical community adjusts to the findings of PEXIVAS, we will need to make decisions about how the data might change our management of critically ill patients with end-organ damage. Given the low incidence of cases in the population, each treatment course will need to be determined on a case-by-case basis, though the options for medical management are currently limited to steroids and immunosuppressive agents. There is a need to develop effective, targeted, and less invasive treatment strategies for this rare but potentially debilitating and devastating diagnosis.