累及气道的复发性多软骨炎27例治疗分析

Clinical characteristics and treatment of 27 patients with airway involvement in relapsing polychondritis

目的分析累及气道的复发性多软骨炎患者的临床特点及治疗转归。方法回顾性分析气道受累的复发性多软骨炎患者的临床资料。结果27例累及气道的复发性多软骨炎患者,女性15例,男性12例,平均年龄52岁(14~65岁),以呼吸道症状起病的23例(85.2%),病程1个月~15年。11例管腔狭窄明显者经保守治疗无效后置入气道内支架,1例支架置入失败,置入术后第2日气促评分1.1±0.3,较治疗前明显缓解(P<0.05),放置支架患者均出现不同程度的肉芽增生、气道再狭窄及呼吸道感染。3例行气管切开术,3例行气管切开及支架置入术。13例管腔狭窄轻者及1例支架置入失败者给予保守治疗,均病情稳定出院,出院时气促评分1.3±0.2,较治疗前缓解(P<0.05)。结论复发性多软骨炎累及气道时,首选内科保守治疗,保守治疗无效时可行支气管镜检查评估管腔狭窄程度,管腔狭窄程度严重者可选择介入治疗,能迅速缓解症状,但并发症较多。

Objective To investigate the clinical characteristics,treatment outcomes in patients with relapsing polychondritis(RP)with airway involvement.Methods The medical records of RP patients with airway involvement were retrospectively analyzed.Results 27 RP patients(15 females,12 males)with airway involvement were included,with median age 52 years old(14~65).Of the 27 patients,23 cases(85.2%)suffered respiratory symptoms in the early stage of the disease,with a history of 1m^15y.11 cases were treated with tracheal stent.One case failed with implantation.The shortness of breath score on the 2nd day after operation was 1.1±0.3,which was significantly relieved(P<0.05).Almost all of the patients with stent were complicated with granulosis,airway restenosis and recurrent respiratory infection.3 cases underwent tracheotomy,and 3 cases with tracheotomy and tracheal stent.13 cases with mild stenosis and 1 case with unsuccessful stent implantation were treated conservatively.All patients were discharged from hospital with stable condition.The shortness of breath score was 1.3±0.2 at discharge,which was significantly different compared with before(P<0.05).Conclusion Conservative medical treatment is the first choice when the airway is involved in RP.Bronchoscopy can be used to assess the degree of lumen stenosis when conservative treatment is ineffective,and interventional therapy can be used to relieve symptoms quickly if stenosis is serious.