近端指间关节周围胶原沉积症13例临床特征分析

Clinical manifestations of pachydermodactyly:an analysis of 13 cases

目的探讨近端指间关节周围胶原沉积症的免疫学改变及临床特点。方法总结1998年3月至2006年1月中国医科大学附属第一医院风湿免疫科收治的13例近端指间关节周围胶原沉积症患者的临床表现、一般实验室检查、免疫学检查和X线检查结果并进行临床分析。结果13例患者中男11例，女2例，发病年龄12～19岁，平均（16±3）岁。临床特点为慢性无痛性近端指间关节周围膨大、肿胀，多呈对称性分布。免疫学检测结果无异常所见。X线检查除软组织肿胀影外，关节及骨质无改变。结论本病属于风湿免疫性疾病，主要应与累及近端指间关节的幼年型类风湿关节炎相鉴别。

Objective To study the immunological changes and clinical features of pachydermodactyly. Methods Thirteen cases of pachydermodactyly were studied and the literature about this disease was reviewed. Results The mean disease onset age was （ 16 ±3 ） years, 11 of whom were male, 2 were female; clinical feature was chronic non-inflammatory bulbous swelling of proximal interphalangeal joints, most of which distributed symmetrically ;there were no abnormal results in immunological examination;X-ray showed only soft tissue swelling with no articular or bone damage. Conclusion Pachydermodactyly belongs to rheumatological diseases, and should be differentiated from other arthritis involving PIP joints.