复发性多软骨炎气道累及的内镜下表现和介入处理

目的分析气道受累的复发性多软骨炎（RP）患者的内镜下表现和气道介入处理。方法前瞻性的收集呼吸道累及的RP患者6例，记录临床资料，进行支气管镜检查，分析镜下表现的病变部位、粘膜炎症情况、气管和支气管软化程度和喉、声门区病变。并根据CT和支气管镜下病变分期和狭窄程度采用处理方法。结果6例患者中3例表现喉、气管和支气管的复合性病变，4例表现为严重的弥漫性气管、支气管软化。在内镜下2例表现为急性期改变，3例表现为牯膜肥厚增生、气道软化、气道动态萎陷，管腔狭窄明显，另Ⅰ例除上述改变外，尚表现为粘膜下纤维组织增生，管腔病变范围极广，极度狭窄。6例患者均给予免疫抑制剂治疗，4例行机械通气患者共置入金属气道支架8个，均在支架置入后1-60d内成功撤机而出院。结论RP的气道表现主要为粘膜充血、肥厚，软骨环模糊不清，分嵴增宽，管腔狭窄。而随着病情反复发作。最终致粘膜肥厚、气管支气管软化，气道动态萎陷，气道广泛狭窄。自膨式金属支架的置入是对重度气道狭窄的主要治疗措施。急性期患者尚需合用免疫抑制剂。

Endoscopic findings and intenvential management of airway involvement in relapsing polychondritis

Objective To analyse endoscopic findings of airway involved in relapsing polychondritis and the management of such airways using selfexpandable metallic stents. Methods We collected medical records of all patients with airway involvement of PIP prospectively. Recording bronchoscopic findings in detail and treat patients with airway stents and other methods according to findings of bronchoecopic, Results 3 of 6 patients had laryngo - tracheobronchial involvement ,4 patients had severe tracheobronchial stenosis and they had mechanical ventilation. Under bronchoscopy , 2 patients showed acute inflammation and 3 patients presented membrane hypertrophy and extensive dynamic collapse and narrowing of the major airway, another one patient was found to have extensive fthrons tissue formation and extreme stenosis in his airway. 8 metallic stents was applied in the 4 patients who had mechanical ventilation and they were successfully weaned off ventilation 1 - 60days after stem placement. Conclusion Under bronchoscopic, main findings are inflammation of the tracheobronchlal tree, hypertrophy membrane ,obscuring of the cartilage, narrowing of the airway. If there are severe findings including extensive malacia of trecheobronchial with dynamic collapse , ventilation support is needed. Self- expandable metallic airway stents is a major method to treat patients with severe airway involement of RP, with combination of immunosuppressive medications .