Clinical immunology (Orlando, Fla.) · 2016
Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective.
СтатьяНазвание и аннотация: Перевод пока не подготовлен
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Jansen Anne, van Deuren Marcel, Miller Joanne, Litzman Jiri, de Gracia Javier, Sáenz-Cuesta Matías, Szaflarska Anna, Martelius Timi, Takiguchi Yuichi, Patel Smita, Misbah Siraj, Simon Anna, Good syndrome Study Group
Аннотация
Good syndrome (GS) or thymoma-associated immunodeficiency, is a rare condition that has only been studied in retrospective case series. General consensus was that GS has a worse prognosis than other humoral immunodeficiencies. In this study, physicians of GS patients completed two questionnaires with a two year interval with data on 47 patients, 499 patient years in total. Results on epidemiology, disease characteristics, and outcome are presented. Mean age at diagnosis was 60years and median follow-up from onset of symptoms was 9years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14years. Survival was reduced compared to age-matched population controls (5-year survival: 82% versus 95%, p=0.008). In this cohort survival was not associated with gender (HR 0.9, 95% CI 0.3-3.0), autoimmune diseases (HR 2.9, 95% CI 0.8-10.1) or immunosuppressive use (HR 0.3, 95% CI: 0.1-1.2).