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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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5380240 | PMC |
http://dx.doi.org/10.1097/MD.0000000000006024 | DOI Listing |
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