Bai Y, Lin Y
PUMC Hospital, CAMS, PUMC, Beijing 100730, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2000 Mar;23(3):155-7.
To describe the clinical manifestation of relapsing polychondritis(RP) and evaluate the medical therapy of RP, especially management of airway stenosis with intervening method.
Retrospective analysis of 5 cases of RP treated in our hospital since 1995 with reviewing of large series of cases in reported literatures.
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.
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.
描述复发性多软骨炎（RP）的临床表现，并评估RP的药物治疗，尤其是采用介入方法治疗气道狭窄的情况。
回顾性分析我院自1995年以来收治的5例RP患者，并复习大量已报道文献中的病例。
RP是一种罕见的多系统疾病，其特征为软骨结构和结缔组织反复发生炎症以及进行性退变。使用皮质类固醇、免疫抑制药物和氨苯砜进行药物治疗无法阻止疾病进展。呼吸道受累会危及生命，需要进行诸如气管切开术、气管插管或气管支气管支架置入等介入治疗，本文介绍了1例通过自膨式金属支架缓解RP广泛气管支气管塌陷所致严重呼吸困难的病例。
根据临床特征诊断RP并早期进行药物治疗很重要，当喉气管支气管受累加重时，应考虑采用正压气道支持和介入方法来预防气道塌陷。
