复发性多软骨炎五例分析

目的　探讨复发性多软骨炎 (RP)的临床特征、治疗方法 ,特别是RP合并严重气道狭窄的介入治疗。方法　结合文献资料回顾分析 5例临床病例。结果　RP是一种以全身多器官软骨结构及结缔组织反复发作性炎症为特征的自身免疫性疾病。目前以皮质类固醇激素、免疫抑制剂及氨苯砜为主的药物治疗常不能有效控制病变发展 ,其中气道软骨受累最危险 ,常需介入性对症治疗如气管造口、气管内插管、支架等。通过气道内可扩张的金属支架安置术成功缓解了一名RP患者因广泛气道软骨塌陷所致的严重呼吸困难。结论　对有特征性临床表现的病例应及早确诊并予药物治疗 ,当出现气道软骨受累时应及时给予压力支持及介入治疗以维持气道开放

Clinical analysis of 5 cases of relapsing polychondritis

Objective To describe the clinical manifestation of relapsing polychondritis(RP) and evaluate the medical therapy of RP, especially management of airway stenosis with intervening method Method Retrospective analysis of 5 cases of RP treated in our hospital since 1995 with reviewing of large series of cases in reported literatures Result RP is a rare multisystem disorder characterized by recurrent inflammation and progressive degeneration of cartilaginous structure and connective tissue Medical treatment with corticosteroids, immunosuppressive drugs and dapsone could not interrupt the progression of the disease Involvement of the respiratory tract is life threatening and intervening therapy such as tracheostomy,endotracheal intubation or tracheobronchial stents is needed, one case of relieving severe dyspnea caused by extensive tracheobronchial collapse in RP with self expanding metallic stents was presented Conclusion It is important to make the diagnosis of RP according to the clinical features and carry out medial treatment at early stage,positive airway pressure support and intervening methods to prevent the airway collapse should be considered when the laryngotracheobronchial involvement aggreviates