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

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 respirat...

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Detalles Bibliográficos
Autores principales: Vanoli, Jennifer, Riva, Marta, Vergnano, Beatrice, D’Andrea, Gabriele, L’Imperio, Vincenzo, Pozzi, Maria Rosa, Grassi, Guido
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5380240/
https://www.ncbi.nlm.nih.gov/pubmed/28353556
http://dx.doi.org/10.1097/MD.0000000000006024
Descripción
Sumario: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.