Koch Ann-Kristin, Brömme Sabine, Wollschläger Bettina, Horneff Gerd, Keyszer Gernot
Department of Internal Medicine I, University Hospital of Martin-Luther University Halle-Wittenberg, Halle Saale, Germany.
J Rheumatol. 2008 Sep;35(9):1882-91. Epub 2008 Aug 1.
To determine the character and frequency of musculoskeletal manifestations and rheumatic symptoms in patients with cystic fibrosis (CF).
Rheumatic symptoms and signs of 70 patients with CF (age 6 to 61 yrs) were determined by interview and clinical assessment. Age and sex-matched healthy volunteers served as a control group. In CF patients, laboratory measures and bone mineral density (BMD) were investigated. The data were correlated with the CF phenotype [Shwachman Score (ShS), Chrispin-Norman Score (ChNS), and pulmonary function tests (PFT)].
The prevalence of joint pain in the CF patients was 12.9%, with a mean duration of 7 days. Swollen joints were found in 4 patients. None fulfilled the criteria for rheumatoid arthritis or connective tissue disease. Adult CF patients complained more often about noninflammatory back pain and myalgia, and demonstrated reduced spine mobility and impaired everyday life functions compared with the controls. Symptomatic CF patients had elevated erythrocyte sedimentation rate and C-reactive protein levels and performed worse on the ShS, ChNS, and PFT than asymptomatic patients. Antibodies against exotoxin A of Pseudomonas aeruginosa and recombinant Aspergillus fumigatus allergen f4 were found more frequently in CF patients with arthralgia. BMD was decreased in adult patients with more severe CF.
In CF patients, the prevalence of rheumatic symptoms increases with age and CF severity. Our data suggest an association of infections with P. aeruginosa and A. fumigatus with the occurrence of rheumatic symptoms. However, no association of CF with definite inflammatory joint or connective tissue diseases was observed, and no CF-specific pattern of musculoskeletal symptoms was seen.
确定囊性纤维化（CF）患者肌肉骨骼表现及风湿症状的特点和频率。
通过访谈和临床评估确定70例CF患者（年龄6至61岁）的风湿症状和体征。年龄和性别匹配的健康志愿者作为对照组。对CF患者进行实验室检查和骨密度（BMD）检测。将数据与CF表型[施瓦克曼评分（ShS）、克里斯平 - 诺曼评分（ChNS）和肺功能测试（PFT）]进行关联分析。
CF患者关节疼痛的患病率为12.9%，平均持续时间为7天。4例患者出现关节肿胀。无人符合类风湿关节炎或结缔组织病的标准。与对照组相比，成年CF患者更常抱怨非炎性背痛和肌痛，脊柱活动度降低，日常生活功能受损。有症状的CF患者红细胞沉降率和C反应蛋白水平升高，在ShS、ChNS和PFT方面比无症状患者表现更差。在有关节痛的CF患者中，抗铜绿假单胞菌外毒素A和重组烟曲霉变应原f4的抗体更常见。成年CF病情较重的患者骨密度降低。
在CF患者中，风湿症状的患病率随年龄和CF严重程度增加。我们的数据表明铜绿假单胞菌和烟曲霉感染与风湿症状的发生有关。然而，未观察到CF与明确的炎性关节或结缔组织病相关，也未发现CF特异性的肌肉骨骼症状模式。
