【摘要】 目的提高对合并呼吸道受累的复发性多软骨炎的认识、早期诊断和处理。方法收集并分析8例呼吸道受累的复发性多软骨炎临床和辅助检查资料。结果8例患者均表现声音嘶哑、进行性呼吸困难。肺功能检查提示不同程度的阻塞性和限制性通气功能障碍,容量-流速曲线显示平台样改变;胸部CT显示不同程度气道狭窄,气管支气管壁增厚和(或)钙化;气管镜检查发现气管-支气管动态狭窄、软骨环消失;均在呼吸道症状出现后6~16个月确诊;5例使用泼尼松治疗(其中3例单用泼尼松,2例合并氨苯砜,1例并用环磷酰胺)后症状缓解,其中2例置金属支架效果满意。非泼尼松治疗3例中2例死亡。结论呼吸道受累是复发性多软骨炎病情严重的临床表现,由于临床表现不典型和医生对该病缺乏认知,容易误诊和漏诊,皮质激素可缓解症状,气管切开、气管支架置入、机械通气能缓解患者呼吸困难。更多还原

【Abstract】 Objective To enhance the understanding of the variety of clinical features and the importance of early diagnosis of relapsing polychondritis(RP).especially the cases with airway involvement as inifial presenting symptom.Methods The clinical features,laboratory studies and prognosis of RP with airway involvement in 8 patients were analyzed retrospectively.Results All eight patients had severe airway involvement as inital presenting manifestation.Spirometry showed severe combined obstructive and restrictive ventilatory defects and a plateau in flow～volume curves;Chest CT showed airway stenosis of different degrees with tracheobronchial wall thickening and/or calcifications in all the cases.Bronchoscopy revealed dynamic collapse of the proximal airways and dissolving of cartilagerings.Diagnosis was made 6 months to 16 months after the initial respiratory symptom of the disease.Pharmacotherapy included prednisone,immunosuppressive agents and dapsone.Self-expansible metallic stents of different sizes were placed in two rases.Of the 8 case,2 died from respiratory tract infection and respiratory failure and one died from suffocation.Conclusion Respiratory tract involvement is the most severe and likely the inifial presenting manifestation of relapsing polychondritis;early diagnosis and treatment with prednisone,immunosuppressive agents and intervening methods can improve the prognosis.更多还原