Vanoli Jennifer, Riva Marta, Vergnano Beatrice, D'Andrea Gabriele, L'Imperio Vincenzo, Pozzi Maria Rosa, Grassi Guido
Divisione 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.
Medicine (Baltimore). 2017 Mar;96(13):e6024. doi: 10.1097/MD.0000000000006024.
Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis affecting small- and medium-sized blood vessels, mostly involving lung and kidney.
We report the case of a 33-year-old man that presented with acute respiratory distress syndrome caused by alveolar hemorrhage.
Aggressive GPA presenting with diffuse alveolar hemorrhage and multiorgan involvement.
Immunosuppressive therapy, plasma exchange, extracorporeal membrane oxygenation (ECMO).
Relapse occurred very early, despite immunosuppressive treatment, with a rare involvement of genital system (epididymitis) and rapidly progressive glomerulonephritis difficult to treat.
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.
肉芽肿性多血管炎(GPA)是一种抗中性粒细胞胞浆抗体(ANCA)相关的血管炎,累及中小血管,主要侵犯肺和肾。
我们报告一例33岁男性患者,因肺泡出血导致急性呼吸窘迫综合征。
侵袭性GPA伴弥漫性肺泡出血和多器官受累。
免疫抑制治疗、血浆置换、体外膜肺氧合(ECMO)。
尽管进行了免疫抑制治疗,但疾病仍很早就复发,累及罕见的生殖系统(附睾炎),并出现难以治疗的快速进行性肾小球肾炎。
GPA是一种具有挑战性、多方面的疾病,可能需要积极的支持治疗,且复发率高,可能出现不常见的受累部位。