THE PREVALENCE AND CLINICAL CHARACTERISTICS OF CYSTIC FIBROSIS ARTHROPATHY

对囊性纤维化关节病（CFA）的患病率、发病率、临床特征、预后特征和病理描述进行前瞻性研究。CFA的特点是反复发作，通常是疼痛的单关节炎或多关节炎。许多患者还表现为红斑结节样皮疹，或在少数病例中表现为紫癜性皮损。关节炎症的发作持续一天到几周。一些CFA患者进展为慢性关节炎，这可能会使人非常虚弱。CFA的适当治疗尚不清楚，也未在大型前瞻性研究中进行研究。该研究同时在儿童医院和麻省总医院进行，是各机构风湿病科和囊性纤维化中心的联合努力。该研究需要通过问卷调查和后续医疗图表审查、体格检查、实验室检查和选择性x射线检查来筛查CFA的存在。该研究旨在提高CFA的临床和基础科学知识，并可能深入了解可能的预防或治疗措施。

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.