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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。
To study the clinical features and diagnosis and treatment of recurrent polychondritis (RPC). Methods: The clinical effects of 11 cases of RPC treated with steroids, immunosuppressive agents such as Prednisone, CTX and DDS during 1990-1997 and followed up for 3 to 6 years were retrospectively analyzed. Results: Nine patients successfully controlled clinical symptoms without recurrence (82%, 9/11), 4 cases with posterior rhinofacial deformity and 1 case with advanced disease. The lesions involved the respiratory tract and the kidneys. Extubation after tracheotomy was difficult and the treatment was ineffective. With tube. Another case of lesions involving the laryngotracheillar cartilage, did not adhere to long-term treatment, disease recurrence and infection, respiratory blockage death. Conclusion: 1 RPC is a rare disease, presumably may be an autoimmune disease involving multiple organs of the cartilage structure and connective tissue, including the ear nose and nose larynx, trachea, joint, kidney and cardiovascular; 2 early diagnosis and treatment of early prognosis is good, delayed diagnosis , The disease is irreversible, the stubborn refractory; 3 lesions involving the respiratory tract poor prognosis, due to laryngeal mucosal edema, granulation, scar, lumen collapse, airway obstruction and fatal; 4 no specific drugs in this disease, the best drug is Steroids and immunosuppressive agents, emphasizing the initial high-dose impact treatment, the importance of long-term low-dose maintenance administration.