Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: A case report

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Abstract

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.

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