复发性多软骨炎临床分析

目的探讨复发性多软骨炎(RP)的临床特点和诊疗方法。方法回顾分析5例病例。结果2例成功控制症状无复发,1例未坚持用药者症状反复发作,1例喉气管支气管软骨炎患者,气管切开术后拔管困难,长期带管,病变复发致气道阻塞死亡,1例失访。此5例患者中2例后遗耳廓和外鼻畸形。结论RP是罕见病,据推测可能是自身免疫性疾病,累及多器官的软骨结构及结缔组织。目前以类固醇激素、免疫抑制剂及氨苯砜为主的药物治疗可控制部分患者病变发展,病变累及呼吸道者预后不良。对有特征性临床表现的病例应及早确诊,给予类固醇及免疫抑制剂是改善预后的关键。

Clinical analysis of relapsing polychondritis

Objective To probe the clinical characteristics,diagnosis and therapy of relapsing polychondritis(RP).MethodsRetrospective analysis of 5 cases with RP treated in our hospital since 1995 with reviewing of large series of cases in reported literatures.Results 2 patients were controlled successfully without recurrence;one patient had symptoms repeatedly;one patient with involvement of laryngo tracheobronchial chondritis suffered from difficult intubation after tracheotomy and died of recurrence.Another patient lost follow-up.There was residual ear or nose deformity in 2 of 5 patients.Conclusion RP is a rare disease of presumed autoimmunology pathogenesis.It is important to make the diagnosis of RP according to the clinical features and carry out medical treatment with corticosteroid and immunosuppressant at early stage can improve the prognosis.