中文摘要：

目的探讨肺朗格汉斯细胞组织细胞增多症（PLCH）合并肺动脉高压（PH）的临床表现，以提高对本病的认识。方法回顾性分析2006年6月至2011年6月首都医科大学附属北京朝阳医院呼吸与危重症医学科收治的11例PLCH患者的临床资料。结果11例PLCH患者中4例合并PH（36％），PLCH—PH临床症状较无PH者重，表现为Borg呼吸困难评分明显增加，杵状指，心功能（NYHA）达Ⅲ～Ⅳ级，并出现右心衰竭体征。胸部HRCT以双上中肺野弥漫囊腔样损害为主，并可见肺动脉增宽，右心增大。肺一氧化碳弥散量及动脉血氧分压也显著降低，并出现呼吸衰竭。彩色多普勒超声心动图示肺动脉收缩压升高。肺活检病理除朗格汉斯细胞浸润及囊腔样改变外，还可见肺小血管管腔狭窄及毛细血管扩张。治疗以氧疗、对症缓解症状为主，3例接受激素或联合免疫抑制剂治疗未见明显效果；4例PLCH—PH，仅1例随访1年病情稳定。结论PH是PLCH比较常见的并发症，是PLCH病情进展、预后不良的标志；对于PLCH患者应注意PH的早期评估与预防。

英文摘要：

Objective To study the clinical features of pulmonary hypertension （PH） complicating pulmonary langerhans＇ cell histiocytosis （PLCH）. Methods Medical records of 11 PLCH patients were reviewed from June 2006 to June 2011. Results 4 of the 11 PLCH patients presented PH with more severe clinical presentations. The major symptoms were laboring dyspnea and diminished exercise capacity （NYHA functional class Ⅲ or Ⅳ）, which were complicated with the sigh of right heart failure. The cystic change and pulmonary artery/main artery 〉 1 were predominate on chest high-resolution computed tomography. Right heart enlargement was also found. As to pulmonary function presentation, the patients displayed severe carbon monoxide diffusing capacity impairment and significant hypoxemia. The pulmonary artery systolic pressure were highly increased. Besides the typical histopathological features presented, involvement of both arteries and veins was also observed. Oxygen and symptomatic therapy were essential in the part of clinical treatment. 3 of the 4 PLCH-PH received corticosteroids or chemotherapeutic agents, but the effect was not ideal. Only one case of PLCH-PH have a stable condition. Conclusions PH is a common complication of PLCH and seems to predict a poor prognosis. It is necessary to derive an early diagnosis and prevention.