侵犯喉气管支气管的复发性多软骨炎

Laryngotracheobronchial involvement in relapsing polychondritis

目的 分析喉、气管和支气管受侵的复发性多软骨炎 (relapsingpolychondritis,RP)临床特点及预后。方法 回顾性分析 13例侵犯喉、气管和支气管的RP患者临床资料。结果 1983年～1998年间我院收治 2 0例RP中 ,13例 (65 % )累及喉、气管和支气管 ,病变范围为弥漫性或局限性 ,病变部位 :喉 (46 % ) ,气管 (69% ) ,支气管 (69% )。治疗 :用皮质激素、免疫抑制剂和氨苯砜控制症状 ,4例因呼吸道梗阻行气管切开术 ,2例气管塌陷的患者用T型管或金属支架治疗。 13例中 2例治疗无效 ,死于呼吸道并发症 ,5年生存率 78 6 %。结论 呼吸道受累是RP病情严重的临床表现及主要死亡原因 ,经皮质激素、免疫抑制剂、氨苯砜治疗或气管切开术后可控制症状 ,但无法控制病程发展。

Objective To investigate the clinical cause, treatments and prognosis of laryngotracheobronchial involvement in relapsing polychondritis (RP) Methods Thirteen patients of RP with laryngotracheobronchial involvement were review retrospectively Results From 1983 to 1998, 20 patients with RP were managed in this hospital Of them, 13 (65%) involving the laryngotracheobronchial region were observed Respiratory tract lesions might be diffuse or localized and occurred in larynx (46%), trachea (69%) and bronchus (69%) Corticosteroids, immunosuppressive agents and dapsone were used in these patients Tracheotomy was performed in 4 patients with airway obstruction Montgomery T tube or self expanding metal stent were used in 2 patients with tracheal collapse Of 13 case, 2 died from respiratory complications The 5 years survival rate was 78 6% Conclusion Respiratory tract involvement is the most severe manifestation of the relapsing polychondritis and the main cause of death despite aggressive medical therapy or tracheotomy