Extracorporeal Membrane Oxygenation Buys Time for Diagnosis and Rescue Lung Transplantation in Severe Isolated Pauci-immune Pulmonary Capillaritis.

Abstract

We present the clinical course and management of a young man affected by catastrophic Isolated pauci-immune pulmonary capillaritis (IPIPC), who required extracorporeal membrane oxygenation (ECMO) for severe acute respiratory distress syndrome (ARDS) and ultimately underwent bilateral lung transplantation (LTx) due to unresolving life-threatening respiratory failure. The patient was admitted to emergency department (ED) due to worsening dyspnea. Bilateral infiltrates were detected on the chest X-ray (CXR) and treatment for community acquired pneumonia was established despite negative microbiological testing. The computed tomography (CT) scan showed bilateral diffuse centrilobular ground glass nodules and lower lobes consolidations. Rapidly worsening hypoxemic respiratory failure required intubation, and after prone positioning failed, VV ECMO support was initiated. Extensive infectious and autoimmune screening resulted negative. No extrapulmonary organ dysfunction developed. Prone positioning and high dose methylprednisolone were ineffective, with persistence of intrapulmonary shunt >60% and intermediate-low respiratory system compliance (25 ml/cmH₂O). Thoracoscopic lung biopsy established the diagnosis of IPIPC. Given the poor prognosis and the severe condition, the patient was enlisted for LTx. Immediately after the patient was weaned from ECMO. After 106 days the patient was discharged in good clinical conditions.