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

JANSEN, Anne, Marcel van DEUREN, Joanne MILLER, Jiří LITZMAN, Javier de GRACIA, Matías SÁENZ-CUESTA, Anna SZAFLARSKA, Timi MARTELIUS, Yuichi TAKIGUCHI, Smita PATEL, Siraj MISBAH and Anna SIMON. Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. Clinical Immunology. San Diego: Academic Press Inc., 2016, vol. 171, "neuvedeno", p. 12-17. ISSN 1521-6616. Available from: https://dx.doi.org/10.1016/j.clim.2016.07.025.

Basic information

• Original name: Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective
• Authors: JANSEN, Anne (528 Netherlands), Marcel van DEUREN (528 Netherlands), Joanne MILLER (826 United Kingdom of Great Britain and Northern Ireland), Jiří LITZMAN (203 Czech Republic, guarantor, belonging to the institution), Javier de GRACIA (724 Spain), Matías SÁENZ-CUESTA (724 Spain), Anna SZAFLARSKA (616 Poland), Timi MARTELIUS (246 Finland), Yuichi TAKIGUCHI (392 Japan), Smita PATEL (826 United Kingdom of Great Britain and Northern Ireland), Siraj MISBAH (826 United Kingdom of Great Britain and Northern Ireland) and Anna SIMON (528 Netherlands).
• Edition: Clinical Immunology, San Diego, Academic Press Inc. 2016, 1521-6616.

Other information

• Original language: English
• Type of outcome: Article in a journal
• Field of Study: 30300 3.3 Health sciences
• Country of publisher: United States of America
• Confidentiality degree: is not subject to a state or trade secret
• Impact factor: Impact factor: 3.990
• RIV identification code: RIV/00216224:14110/16:00090643
• Organization unit: Faculty of Medicine
• Doi: http://dx.doi.org/10.1016/j.clim.2016.07.025
• UT WoS: 000386195100002
• Keywords in English: Immunodeficiency; Thymoma; Infection; Good syndrome
• Tags: EL OK
• Tags: International impact, Reviewed

Abstract

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).