Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis affecting small- and medium-sized blood vessels, mostly involving lung and kidney.Patient concerns:
We report the case of a 33-year-old man that presented with acute respiratory distress syndrome caused by alveolar hemorrhage.Diagnoses:
Aggressive GPA presenting with diffuse alveolar hemorrhage and multiorgan involvement.Inteventions:
Immunosuppressive therapy, plasma exchange, extracorporeal membrane oxygenation (ECMO).Outcomes:
Relapse occurred very early, despite immunosuppressive treatment, with a rare involvement of genital system (epididymitis) and rapidly progressive glomerulonephritis difficult to treat.Lessons:
GPA is a challenging, multifaceted disease that can require aggressive supportive therapy and is associated with a high rate of relapse that may present with uncommon site of involvement.