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Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane
Jennifer Vanoli1, Marta Riva, Beatrice Vergnano
1Divisione di Clinica Medica Divisione di Anestesia e Rianimazione Divisione di Radiologia Divisione di Anatomia Patologica, Ospedale San Gerardo, Monza University of Milano-Bicocca and Istituto di Ricerca a Carattere Scientifico IRCCS Multimedica, Sesto San Giovanni, Milano, Italy.
Rationale:
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.
