摘要目的 探讨复发性多软骨炎的临床表现、治疗和预后,以提高复发性多软骨炎的诊治水平.方法 回顾性分析48例复发性多软骨炎患者的临床资料.结果 48例患者中,男30例,女18例,确诊时年龄11~72(44±14)岁.受累器官包括呼吸系统23例(47.9%),耳部18例(37.5%),关节17例(35.4%),眼部12例(25.0%),鼻部8例(16.7%),皮肤3例(6.25%).行肺CT检查23例,鼻窦及喉部CT 3例,胸部 MRI 2例;行肺功能检查10例;行气管镜检查10例;行气管镜下黏膜活检6例.48例患者中,11例患者仅给予对症治疗,不使用糖皮质激素;37例接受糖皮质激素治疗,其中19例同时加用了免疫抑制剂,2例应用生物制剂;因疗效不佳1例气管切开,1例应用无创呼吸机,1例支架植入术,余34例患者治疗后病情好转.结论 复发性多软骨炎易累及全身多个系统,误诊率高,应用糖皮质激素联合免疫抑制剂治疗效果较佳,但呼吸系统受累导致气道严重狭窄者可能需要气管切开及气管支架治疗.

abstractsObjective To investigate relapsing polychondritis clinical manifestations, treatment and prognosis to improve the diagnosis and treatment of it.Methods The clinical data of 48 patients with relapsing polychondritis disease were retrospectively analyzed.Results Among the 48 patients, there were 30 male patients and 18 female patients, which diagnosed at age 11 to 72 (44±14) years old.The affected organs including 23 cases of the respiratory system (47.9%), 18 cases of ear (37.5%), 17 cases of joint (35.4%), 12 cases of ocular (25.0%), 8 cases of nasal (16.7%), 3 cases of skin (6.25%).Twenty-three cases of pulmonary CT examination, 3 cases of Sinus and throat CT, 2 cases of chest MRI;10 cases of pulmonary function examination;10 cases of underwent bronchoscopy;6 cases of tracheal endoscopic mucosal biopsy.In 48 patients, 11 cases were given symptomatic treatment;37 patients received glucocorticoid therapy, among them 2 cases combined with immunosuppressive agents, 2 cases of application of biological agents;1 case of tracheotomydue to poor efficacy, 1 case of application of noninvasive ventilation, 1 case of stent implantation, remaining 34 cases improvement of the disease after treatment.Conclusions Relapsing polychondritis is easily multisystemic and has a high misdiagnosis rate.Therapeutic effect is better with corticosteroids and immunosuppressant, but the respiratory system involvement leads to serious airway stenosis may require a tracheotomy and tracheal stents.