伴气管狭窄的复发性多软骨炎3例

A case study of relapsing polychondritis with the involvement of the respiratory tract

目的探讨复发性多软骨炎(RP)的临床特点及诊治方法。方法分析3例复发性多软骨炎患者的临床资料、相关检查结果及治疗方法 ,并结合文献复习。结果 3例病理检查确诊的RP患者均有典型临床表现,内镜、CT三维重建等检查可明确病变范围和严重程度,均予糖皮质激素及免疫抑制剂治疗,症状早期均有不同程度改善,但症状反复。1例复发后接受喉气管成型支架扩张术,随访至今病情稳定;1例于诊断后2年死亡;1例5年后死亡。结论 RP是一种少见的原因不明的累及全身系统的疾病,诊断需结合临床表现、CT、气管镜检查及病理检查等。糖皮质激素及免疫抑制剂能在早期部分缓解患者的症状,气管切开及狭窄气道重建能较好地解除呼吸道梗阻,延长生存时间,但气道受累的RP预后较差。

Objective To investigate the clinical characteristics of relapsing polychondritis（RP） with the involvement of the respiratory tract.Methods The clinical features,tests and treatment of RP with airway involvement in 3 patients were retrospectively reviewed.Results Three RP cases of typical symptoms were confirmed by biopsy.CT scan with 3D reconstruction and bronchoscopy revealed the range and severity of the lesion.After initial medications,such as corticosteroids and immunosuppressive drugs,the symptoms in all cases were significantly alleviated for a while but tended to recur and deteriorate later.During the follow-up,one recurrent case adopted laryngotracheal reconstruction in which pedicled surgical flaps were applied and a long-term stable condition persisted.The other 2 cases died in the second and fifth year after the diagnosis.Conclusion RP with airway involvement is a rare disease affecting multiple systems in the body.Diagnosis is made on the clinical features,imaging studies and pathological examination.Early treatment with corticosteroids and immunosuppressive agents might achieve a relatively satisfying result at the beginning.However,in the later stage,invasive approaches,including tracheotomy and laryngotracheal reconstruction,should be applied for a better prognosis.