复发性多软骨炎累及气道二例并文献复习

目的探讨复发性多软骨炎累及气道的诊断及治疗方法。方法报道2例累及气道的复发性多软骨炎患者的临床资料,总结其诊断和治疗经验,并复习相关文献。结果 2例患者均为老年男性,临床表现为咳嗽、气促、发热等,在基层医院均被误诊。肺功能示阻塞性通气功能障碍。吸气相胸部CT扫描可显示受累气道的形态异常：大气道管壁弥漫性增厚伴管腔狭窄,大气道软骨区的钙化;以气管前、侧壁软骨部增厚为主,后壁的膜部多不受累。呼气相胸部CT扫描可显示气管支气管的功能异常,管壁软化塌陷。2例患者经单用糖皮质激素或糖皮质激素联合免疫抑制剂治疗后病情改善。结论复发性多软骨炎致气道狭窄患者容易误诊,胸部CT能很好地显示气道壁的变化,对本病的诊断有重要价值。糖皮质激素或糖皮质激素联合免疫抑制剂治疗能改善患者病情。

Airway Involvement in Relapsing Polychondritis:Clinical Analysis of Two Cases and Literature Review

Objective To explore the diagnosis and treatment of airway involvement in relapsing polychondritis.Methods The clinical data of two patients with relapsing polychondritis with airway involvement were reported and the relative literatures were reviewed.Results The two patients were both old males,with clinical manifestations of cough,dyspnea,and fever.They were misdiagnosed in a other hospital.The pulmonary function tests showed obstructive ventilatory impairemnt.On inspiratory CT,tracheal/tracheobronchial wall thickening and airway stenosis,with or without tracheal cartilage calcification were common findings.The tracheal cartilages thickeness and membranous wall were normal.On expiratory CT scans,functional abnormalities were identified such as tracheobronchomalacia.The patients were relieved by medication of corticosteroids or with immunodepressant.Conclusions The relapsing polychondritis with airway involvement is easy to be misdiagnosed.Chest CT examination is a valuable method for diagnosis of relapsing polychondritis.Corticosteroids and immunodepressant can improve the outcome.