[Clinical characteristics and prognosis of mediastinal fibrosis].

Objective: To investigate the clinical characteristics and prognosis of mediastinal fibrosis. Methods: Twelve patients with mediastinal fibrosis diagnosed between 2008 and 2015 in our hospital were studied retrospectively. Clinical manifestations, radiological characteristics, endoscopic features, treatment and prognosis were analyzed. Results: There were 3 males and 9 females, with a mean age of 68.8 years.Six patients had previous tuberculosis infection. The most common clinical symptoms were dyspnea on exertion (11 cases), cough (7 cases), and wheezing (6 cases). Chest CT scans revealed an infiltrative mediastinal process, with a discrete mass, enlargement of mediastinal lymph nodes, mediastinal lymph node calcification (9 case). Twelve patients had bronchial and pulmonary artery compression at lobar or segmental levels, 7 cases had localized pulmonary edema, and 6 cases had pulmonary atelectasis. The principal findings of bronchoscopy were distortion of bronchus with stenosis, multiple pigmentation of bronchial mucosa, and bronchial mucosal edema. Pulmonary hypertension (PH) was the main severe complication. One patients suffered from sudden death after bronchoscopy. Eleven patients were followed for 3 month to 7 years, and 5 patients got progression. Anti-tuberculosis therapy with or without corticosteroid was not beneficial. Conclusion: Tuberculosis was the leading cause of mediastinal fibrosis in our study, which was characterized with diffuse bronchial and pulmonary artery compression at lobar or segmental levels, and multiple pigmentation of bronchial mucosa.Anti-tuberculosis therapy with or without corticosteroids was not beneficial.目的： 探讨纵隔纤维化的患者的临床特点及预后。 方法： 回顾性分析北京大学第一医院2008年至2015年确诊的12例纵隔纤维化患者，其中男3例，女9例，年龄55～86岁，平均68.8岁，总结其临床表现、影像学特点、气管镜下表现和预后。 结果： 患者主要临床症状为呼吸困难(11例)、咳嗽(7例)和喘息(6例)。影像学表现为纵隔不定型软组织影，伴或不伴淋巴结肿大，钙化(9例)，继发多叶、段的支气管及肺动脉狭窄(12例)，肺静脉狭窄(6例)，肺不张(6例)和不对称肺水肿(7例)。气管镜下可见弥漫叶、段支气管扭曲、狭窄，多发黏膜色素沉着、水肿，1例患者于支气管镜术后猝死，主要并发症为肺动脉高压。11例患者进行了随访，随访时间3个月至7年，5例病情进展，抗结核及糖皮质激素治疗无效。 结论： 本组患者纵隔纤维化以多发叶、段肺血管和气道受累狭窄，支气管黏膜色素沉着为特征，部分患者抗结核及合用糖皮质激素治疗无效。.