耳鼻喉气管支气管的复发性多软骨炎(附11例报告)

EAR NOSE AND LARYNGOTRACHEOBRONCHIAL OF RELAPSING PO LYCHONDRITIS ( A REPORT OF 11 CASES)

研究复发性多软骨炎 (RPC)的临床特点和诊疗方法。方法 :回顾分析 1990～ 1997年间用类固醇、免疫抑制剂如Prednisone,CTX及 DDS治疗 ,随访 3～ 6年的 11例 RPC的临床效果。结果 :9例成功控制临床症状无复发 (82 % ,9/ 11) ,4例后遗耳鼻畸形 ,1例晚期患者 ,病变累及呼吸道及肾脏 ,气管切开术后拔管困难 ,治疗无效 ,长期带管。另 1例病变累及喉气管软骨 ,未坚持长期治疗 ,病变复发合并感染、呼吸道阻塞死亡。结论 :1RPC是罕见病 ,据推测可能是自身免疫性疾病 ,累及多器官的软骨结构及结缔组织 ,包括耳鼻眼喉、气管、关节、肾脏及心血管 ;2早诊早治预后良好 ,延误诊断 ,病为不可逆性 ,则顽固难愈 ;3病变累及呼吸道者预后不良 ,因喉气管粘膜水肿 ,肉芽 ,疤痕 ,管腔塌陷 ,气道阻塞而致命 ;4本病无特效药物 ,最好药物是类固醇及免疫抑制剂 ,强调初期大剂量冲击治疗 ,长期低剂量维持给药的重要性。

Objective:To study the clinical characteristic diagnosis and therapy of relapsing polychondritis(RPC) Methods:A 3~6 year's follow up from 1990 to1997 of 11 patients with PRC treated with steroid,immunosuppressor such as prednisone Cyclophosphamid (CTX) and Dapsone were reviewed and analyzed Results:9 patients were controlled successfully without recurrence (82%,9/11);4 of 11 patients have residual ear or nose deformity One patient with involvement of laryngeal trachea and cardiovascular died of recurrent and complicated infection of respiratory tract due to not receiving program of long term therapy Another patient is on advanced stage with involvement of laryngeal trachea and kidney that have not response to long term therapy,and after tracheotomy suffered from difficult extubation and refractory Conclusions:①RPC is a rare disease of presumed autoimmunology pathogenesis It may involve multiple organ system of cartilaginous structure and connective tissue,including ear,nose,eye,laryngeal trachea,articulation,kidney and cardiovascular;②Early diagnosis is very important with excellent prognosis Delay in diagnosis for advanced stage becomes unrecoverable with refractory;③The prognosis of laryngeal trachea involvement is very poor,heavy dyspnea caused by respiratory tract of edema,granuloma,scarring and collapse results in respiratory obstruction,and the outcome is fatal;④There are not specific effective drugs for RPC;the most effective drug is corticosteroid and immunosuppressor The importance of high dose in initial stage and long term steroid therapy of low dose therapy is stressed