Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective

Autoři	JANSEN Anne DEUREN Marcel van MILLER Joanne LITZMAN Jiří GRACIA Javier de SÁENZ-CUESTA Matías SZAFLARSKA Anna MARTELIUS Timi TAKIGUCHI Yuichi PATEL Smita MISBAH Siraj SIMON Anna
Rok publikování	2016
Druh	Článek v odborném periodiku
Časopis / Zdroj	Clinical Immunology
Fakulta / Pracoviště MU	Lékařská fakulta
Citace
Doi	http://dx.doi.org/10.1016/j.clim.2016.07.025
Obor	Epidemiologie, infekční nemoci a klinická imunologie
Klíčová slova	Immunodeficiency; Thymoma; Infection; Good syndrome
Popis	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 60 years and median follow-up from onset of symptoms was 9 years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14 years. 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).