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.

Vanoli, J., Riva, M., Vergnano, B., D'Andrea, G., L'Imperio, V., Pozzi, M., et al. (2017). Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: A case report. MEDICINE, 96(13) [10.1097/MD.0000000000006024].

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

Vanoli, J
;
Riva, M;Vergnano, B;L'imperio, V;Pozzi, Mr
;
Grassi, G.
2017

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.
Articolo in rivista - Articolo scientifico
Case report, Diffuse alveolar hemorrhage, Extracorporeal membrane oxygenation, Granulomatosis with polyangiitis
English
Vanoli, J., Riva, M., Vergnano, B., D'Andrea, G., L'Imperio, V., Pozzi, M., et al. (2017). Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: A case report. MEDICINE, 96(13) [10.1097/MD.0000000000006024].
Vanoli, J; Riva, M; Vergnano, B; D'Andrea, G; L'Imperio, V; Pozzi, M; Grassi, G
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/175197
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