复发性多软骨炎48例临床特征及治疗效果分析

Clinical characteristic and treatment effect of 48 patients with relapsing polychondritis

目的分析复发性多软骨炎的临床特征及治疗效果。方法回顾性分析2000年9月-2014年11月在解放军总医院住院并确诊的复发性多软骨炎患者48例的临床资料。结果48例患者中,男22例,女26例,确诊时年龄15-79（46.7±13.9）岁。从首次就诊到确诊中位时间为5个月。受累器官包括关节33例（68.8%）,耳部31例（64.6%）,呼吸系统29例（60.4%）,眼部26例（54.2%）,鼻部20例（41.7%）,皮肤9例（18.8%）,血液系统9例（18.8%）,肾脏5例（10.4%）。在呼吸系统受累的29例患者中,行肺CT检查22例,表现为气管及主支气管壁增厚15例（68.2%）,气道狭窄14例（63.6%）,气道壁钙化8例（36.4%）,气道塌陷2例（9.1%）;行喉CT检查6例,喉部软组织增厚2例,声门下气管狭窄、管壁增厚2例,喉腔闭塞1例。48例全部接受糖皮质激素治疗,其中40例同时加用了免疫抑制剂,4例因疗效不佳行气管切开,余44例治疗后病情好转。随访30例,其中4例因喘息、气短及肺炎多次住院治疗,1例因呼吸闲难加重行气管支架治疗,术后出现呼吸衰竭,其余25例病情平稳。结论复发性多软骨炎易累及全身多个系统,误诊率较高,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。

Objective To analyze the clinical characteristics and therapeutic effect of relapsing polychondritis. Met-hods Retrospective analysis from 2000 September to 2014 November clinical data of 48 patients with relapsing polychondritis in PLA General Hospital. Results In the 48 patients, male were 22 cases, female were 26 cases, age at diagnosis was 15-79 （46. 7 ± 13. 9） years old. From the first visits to the diagnosis with a median of 5 months. The involved organs including the joint in 33 cases （68. 8%）, ears in 31 cases （64. 6%）, 29 cases of respiratory tract （60. 4%）, 26 cases of eyes （54. 2%）, 20 cases of nasal （41. 7%）, 9 cases （18. 8%） in skin, 9 cases of blood system （18. 8%）, and kidney in 5 ca-ses （10. 4%）. In 29 cases of respiratory system involvement, 22 cases received lung CT scan, 15 cases revealed the tracheal and bronchial wall thickening （68. 2%）, airway stenosis in 14 cases （63. 6%）, airway wall calcification in 8 cases （36. 4%）, upper airway collapse in 2 cases （9. 1%）;6 cases received laryngeal CT examination, throat soft tissue thicken-ing in 2 cases,subglottic and tracheal stenosis, wall thickening in 2 cases,laryngeal cavity occlusion in 1 cases. All 48 cases received corticosteroid therapy, of which 40 cases combined with immune inhibitor, tracheotomy was performed in 4 cases be-cause of poor efficacy, the remaining 44 cases after treatment, the condition was improved. Follow up of 30 cases, 4 cases re-admitted to hospital several times because of wheezing, shortness of breath and pneumonia, in 1 cases received tracheal stent treatment because of dyspnea, respiratory failure occurred after operation, the other 25 cases with stable condition during fol-low up. Conclusion The relapsing polychondritis involves multiple system with a high rate of misdiagnosis, treatment effect of glucocorticoid combined with the application of immunosuppressant are good, but the respiratory system involvement leads to severe airway stenosis may need tracheotomy and tracheal stent.