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

被引：42

作者：

Jansen, Anne ^{[1
]}

van Deuren, Marcel ^{[1
]}

Miller, Joanne ^{[2
]}

Litzman, Jiri ^{[3
]}

de Gracia, Javier ^{[4
]}

Saenz-Cuesta, Matias ^{[5
]}

Szaflarska, Anna ^{[6
,7
]}

Martelius, Timi ^{[8
,9
]}

Takiguchi, Yuichi ^{[10
]}

Patel, Smita ^{[2
]}

Misbah, Siraj ^{[2
]}

Simon, Anna ^{[1
]}

机构：

[1] Radboud Univ Nijmegen, Med Ctr, NCIA, Dept Internal Med, Nijmegen, Netherlands

[2] John Radcliffe Hosp, Dept Immunol, Oxford, England

[3] Masaryk Univ, St Annes Univ Hosp, Dept Clin Immunol & Allergol, Fac Med, Brno, Czech Republic

[4] Univ Autonoma Barcelona, CIBER Ciberes, Dept Pulm Dis, Barcelona, Spain

[5] Donostia Univ Hosp, Biodonostia Hlth Res Inst, San Sebastian, Spain

[6] Jagiellonian Univ, Coll Med, Dept Clin Immunol & Transplantol, Krakow, Poland

[7] Children Univ Hosp, Krakow, Poland

[8] Univ Helsinki, Dept Infect Dis, Helsinki, Finland

[9] Helsinki Univ Hosp, Helsinki, Finland

[10] Chiba Univ, Dept Med Oncol, Chiba, Japan

来源：

CLINICAL IMMUNOLOGY | 2016年 / 171卷

关键词：

Immunodeficiency; Thymoma; Infection; Good syndrome; ADULT-POPULATION; REFERENCE VALUES; TACI MUTATION; HYPOGAMMAGLOBULINEMIA; INFECTIONS; DISORDERS; PATIENT;

D O I：

10.1016/j.clim.2016.07.025

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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). (C) 2016 The Authors. Published by Elsevier Inc.

引用

页码：12 / 17

页数：6

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