呼吸道受累复发性多软骨炎患者的临床特点

Clinical characteristics of 149 relapsing polychondritis patients with respiratory tract involvement

目的探讨复发性多软骨炎(relapsing polychondritis,RP)呼吸道受累的临床特点。方法对2005年12月至2019年9月在北京协和医院住院的RP患者的临床资料进行回顾性分析,重叠其他疾病可影响分析结果者予以排除。结果最终193例RP患者纳入本研究。呼吸道受累RP患者为149例(77.2%),其中气管壁增厚最常见,为123例(82.6%);支气管软化100例(67.1%),气管软化80例(53.7%),声门下狭窄28例(18.8%)。呼吸道受累RP患者较非呼吸道受累RP患者发病更年轻[(43.3±11.1)岁vs.(49.9±14.6)岁,P=0.014],诊断延迟时间[中位数(四分位间距):8(4~14)月vs.5(2.25~11)月,P=0.044]更长,更容易出现胸肋软骨受累。结论呼吸道受累的RP患者发病早,不易诊断,更常出现胸肋软骨受累。

Objective To investigate the clinical characteristics of relapsing polychondritis(RP)patients with respiratory tract involvement.Methods The clinical records of RP patients,hospitalized at Peking Union Medical College Hospital between December 2005 and September 2019,were retrospectively reviewed.Cases overlapped with other diseases that may interfere with the results were excluded.ResultsOf 193 RP patients who were included,149 patients(77.2%)presented respiratory tract involvement.Among them,tracheal thickening was the most common in 123 cases(82.6%),followed by bronchomalacia(100,67.1%),tracheomalacia(80,53.7%),and subglottic stenosis(28,18.8%).Compared with RP patients without respiratory tract involvement,those with respiratory tract involvement were younger at the time of disease onset[(43.3±11.1)years vs.(49.9±14.6)years,P=0.014],experienced the longer time of diagnosis delay[median(IQR),8(4-14)months vs.5(2.25-11)months,P=0.044]and had a higher incidence of thoracic costal cartilage involvement.Conclusion RP patients with respiratory tract involvement were more likely occurred to younger people with delayed diagnosis and thoracic costal cartilage involvement.