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
Nijmegen Center for Immunodeficiency and Autoinflammation (NCIA), Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands.
Nijmegen Center for Immunodeficiency and Autoinflammation (NCIA), Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands.
Clin Immunol. 2016 Oct;171:12-17. doi: 10.1016/j.clim.2016.07.025. Epub 2016 Aug 4.
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).
古德综合征（GS）或胸腺瘤相关免疫缺陷，是一种仅在回顾性病例系列中得到研究的罕见病症。普遍的共识是，GS的预后比其他体液免疫缺陷更差。在本研究中，GS患者的医生在两年的间隔期内完成了两份问卷，涉及47名患者的数据，总计499患者年。文中呈现了关于流行病学、疾病特征和结局的结果。诊断时的平均年龄为60岁，从症状出现开始的中位随访时间为9年。由包膜菌引起的呼吸道感染发生率很高。中位生存期为14年。与年龄匹配的人群对照组相比，生存期缩短（5年生存率：82%对95%，p=0.008）。在该队列中，生存期与性别（风险比0.9，95%置信区间0.3 - 3.0）、自身免疫性疾病（风险比2.9，95%置信区间0.8 - 10.1）或免疫抑制剂的使用（风险比0.3，95%置信区间：0.1 - 1.2）均无关。
