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囊性纤维化相关关节炎的临床表现及危险因素。

Clinical manifestations and risk factors of arthropathy in cystic fibrosis.

机构信息

Department of Pediatric Pneumology, Immunology and Intensive Care Medicine, CF Center / Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.

Department of Pediatric Pneumology, Immunology and Intensive Care Medicine, Pediatric Rheumatology Center / Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.

出版信息

Respir Med. 2019 Feb;147:66-71. doi: 10.1016/j.rmed.2019.01.003. Epub 2019 Jan 17.

DOI:10.1016/j.rmed.2019.01.003
PMID:30704701
Abstract

BACKGROUND

Recurrent joint pain is frequently observed in patients with CF and can lead to reduced activity and quality of life. We conducted this observational study to assess the clinical manifestations, frequency, and risk factors of CF associated arthropathy.

METHODS

Clinical data were collected using a digital quality management system, medical records, and by conducting structured interviews. Univariate and multivariate statistical analysis were performed for statistical interpretation.

STUDY DESIGN

retrospective observational study including 186 patients.

RESULTS

Of 186 patients (Demographics: Mean age 27 years, female gender 104/186 (57%), CFTR F508del homozygous 82/186 (44%) included in the study, 54/186 (29%) had experienced joint symptoms. Joint pain and swelling were the most frequent symptoms. The joints of the hands (JOH) followed by the joints of the feet were most affected. No specific pattern of autoantibodies was discovered. The level of total serum IgG, age, female gender, and pulmonary exacerbations per year were significant risk factors for arthropathy in the study cohort.

CONCLUSIONS

Joint symptoms in CF are a frequent and clinically relevant phenomenon with a distinct clinical pattern. Pulmonary exacerbations and elevated levels of total serum IgG may reflect chronic inflammation in patients with CF and may lead to a specific arthropathy associated with this condition.

摘要

背景

CF 患者常出现反复发作的关节痛,可导致活动减少和生活质量下降。我们进行了这项观察性研究,以评估 CF 相关关节病的临床表现、频率和危险因素。

方法

使用数字质量管理系统、病历和进行结构化访谈收集临床数据。采用单变量和多变量统计分析进行统计解释。

研究设计

包括 186 例患者的回顾性观察性研究。

结果

在 186 例患者中(人口统计学:平均年龄 27 岁,女性 104/186(57%),CFTR F508del 纯合子 82/186(44%)纳入研究,54/186(29%)有过关节症状。关节痛和肿胀是最常见的症状。手部关节(JOH)受累最常见,其次是足部关节。未发现特定的自身抗体模式。总血清 IgG 水平、年龄、女性、每年肺部恶化次数是研究队列中发生关节病的显著危险因素。

结论

CF 中的关节症状是一种常见且具有临床相关性的现象,具有独特的临床模式。肺部恶化和总血清 IgG 水平升高可能反映 CF 患者的慢性炎症,可能导致与该疾病相关的特定关节炎。

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