期刊
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
卷 134, 期 3, 页码 655-+出版社
MOSBY-ELSEVIER
DOI: 10.1016/j.jaci.2014.04.014
关键词
Chronic granulomatous disease; inflammation; primary immunodeficiency; granuloma; inflammatory bowel diseases; macrophage; granulomatous; interstitial lung disease
资金
- French Ministry of Health
- French Association of Patients with Primary Immunodeficiencies (IRIS: Immuno-deficience primitive-recherche-information-soutien)
- LFB
- CSL Behring
- Baxter Biosciences
- GSK
- Pfizer
- Octapharma
- Orphan-Europe
Background: Chronic granulomatous disease (CGD) is a rare phagocytic disorder that results in not only infections but also potentially severe inflammatory manifestations that can be difficult to diagnose and treat. Objective: To describe inflammatory manifestations in a single-center cohort of patients with CGD. Methods: Medical records of patients treated at Necker-Enfants Malades Hospital (Paris, France) between 1968 and 2009 and registered at the French National Reference Center for Primary Immunodeficiencies (CEREDIH) were retrospectively reviewed. Results: In a study population of 98 patients, a total of 221 inflammatory episodes were recorded in 68 individuals (69.4%). The incidence rate of inflammatory episodes was 0.15 per person-year (0.18 in patients with X-linked [XL] CGD and 0.08 in patients with autosomal-recessive [AR] CGD). The most commonly affected organs were the gastrointestinal tract (in 88.2% of the patients), lungs (26.4%), the urogenital tract (17.6%), and eyes (8.8%). Inflammation at other sites (the skin, central nervous system, and tympanum) and autoimmune manifestations (lupus, arthritis, etc) were recorded in 19.1% and 10.3% of the patients, respectively. Granuloma was found in 50% of the 44 histological analyses reviewed. The risk of inflammatory episodes was 2-fold higher in patients with XL-CGD than in patients with AR-CGD (relative risk, 2.22; 95% CI, 1.43-3.46). Conclusions: Patients with XL-CGD have a higher risk of developing inflammatory episodes than do patients with AR-CGD. Although the most commonly affected organ is the gastrointestinal tract, other sites can be involved, making the management of patients with CGD a complex, multidisciplinary task.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据