复发性多软骨炎的诊断和治疗

目的探讨复发性多软骨炎的诊断和治疗方法。方法回顾性分析24例复发性多软骨炎患者的临床表现、诊断和治疗方法。24例患者均行自身抗体检查;6例喉部受累者中4例行喉镜检查;19例下呼吸道受累者中12例行支气管镜检查;16例行受累软骨活检组织病理检查。结果抗核抗体阳性5例、抗SSA抗体阳性2例、抗核RNP抗体阳性2例、抗Sm抗体阳性2例。喉镜检查显示声带水肿、喉管狭窄;支气管镜检查显示气管及左右主支气管狭窄和气管支气管软骨消失;病理检查显示慢性软骨炎症。治疗包括强的松、免疫抑制剂,11例呼吸道症状严重者行喉、气管扩张术。23例患者临床治愈或缓解,1例死亡。结论复发性多软骨炎可累及软骨及结缔组织,以呼吸道破坏最严重,皮质类固醇激素联合免疫抑制剂治疗有效,喉、气管狭窄及塌陷者辅以喉、气管扩张术以改善呼吸道阻塞症状。

Diagnosis and treatment of relapsing polychondritis

Objective To explore the diagnosis and treatment of relapsing polychondritis(RP).Methods The clinical manifestations,diagnosis,and treatment of 24 patients with RP were retrospectively analyzed.Twenty-four RP patients were examined for autoimmune antibody.Laryngoscopy was performed in 4 out of 6 RP patients with laryngeal involvement.Bronchoscopy was performed in 12 out of 19 RP patients with lower respiratory tract involvement.Biopsies were taken from the cartilage involved in 16 out of 24 patients.Results Five patients were ANA positive at present,2 patients were SSA antibody positive,2 patients were anti-RNP antibody positive,and 2 patients were anti-Sm antibody positive.Laryngoscopy revealed vocal fold edema and laryngeal stenosis.Bronchoscopy showed stenosis of trachea and both main bronchi and destruction of tracheobronchial cartilage.Histopathology revealed chronic inflammation of cartilage.Treatment included immunosuppressants(cyclophosphamide,methotrexate)together with prednisone.Eleven patients with respiratory tract involvement had laryngotracheostomy or airway stenting.Twenty-three patients alleviated after treatment,and 1 patients died.Conclusions RP involves cartilage and connective tissue.Laryngotracheobronchial complications are the most severe manifestations of this disease.Corticosteroids and immunosuppressive drugs are effective treatment options.Patients with laryngotracheal stenosis and collapsed tracheobronchial wall should receive laryngostomy,tracheostomy,or airway stenting to improve airway obstruction symptoms.