THE PREVALENCE AND CLINICAL CHARACTERISTICS OF CYSTIC FIBROSIS ARTHROPATHY

囊性纤维性关节病(CFA)的患病率、发病率、临床特征、预后特征和病理描述的前瞻性研究。CFA的特点是反复发作，通常是疼痛的单发性或多发性关节炎。许多患者还表现为结节红斑样皮疹，或少数病例出现紫癜性皮损。关节炎症的症状持续一天到几周。一些患有CFA的患者进展为慢性关节炎，可能会相当虚弱。CFA的适当治疗尚不清楚，也没有在一项大型前瞻性研究中进行研究。这项研究同时在儿童医院和马萨诸塞州综合医院进行，两家医院的风湿科和囊性纤维化中心共同努力。这项研究包括通过问卷筛查CFA的存在，以及后续的病历复查、体检、实验室测试和选择性X光检查。这项研究旨在促进CFA的临床和基本科学知识，并可能获得对可能的预防或治疗措施的洞察。

A prospective study of the prevalence, incidence, clinical characteristics, prognostic features, and pathologic description of cystic fibrosis arthropathy (CFA). CFA is characterized by recurrent, often painful bouts of mono-or polyarthritis. Many patients also manifest an erythema-nodosum-like rash, or in a few cases, purpuric skin lesions. The flares of joint inflammation last from one day to several weeks. Some patients with CFA progress to a chronic arthritis that can be quite debilitating. The appropriate treatment of CFA is not known and has not been studied in a large prospective study. The study is being performed simultaneously at Children's Hospital and The Massachusetts General Hospital as a combined effort of the Rheumatology Departments and Cystic Fibrosis Centers at each institution. The study entails screening for the presence of CFA with a questionnaire and follow-up medical chart review, physical exam, laboratory tests and selective radiographs. The study seeks to advance both the clinical and basic scientific knowledge of CFA and possibly gain insight into possible preventive or treatment measures.