CAJ | 학술논문

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.
목적 탐토폐랑격한사세포조직세포증다증(PLCH)합병폐동맥고압(PH)적림상표현,이제고대본병적인식.방법 회고성분석2006년6월지2011년6월수도의과대학부속북경조양의원호흡여위중증의학과수치적11례PLCH환자적림상자료.결과 11례PLCH환자중4례합병PH(36％),PLCH-PH림상증상교무PH자중,표현위Borg호흡곤난평분명현증가,저상지,심공능(NYHA)체Ⅲ～Ⅳ급,병출현우심쇠갈체정.흉부HRCT이쌍상중폐야미만낭강양손해위주,병가견폐동맥증관,우심증대.폐일양화탄미산량급동맥혈양분압야현저강저,병출현호흡쇠갈.채색다보륵초성심동도시폐동맥수축압승고.폐활검병리제랑격한사세포침윤급낭강양개변외,환가견폐소혈관관강협착급모세혈관확장.치료이양료、대증완해증상위주,3례접수격소혹연합면역억제제치료미견명현효과;4례PLCH-PH,부1례수방1년병정은정.결론 PH시PLCH비교상견적병발증,시PLCH병정진전、예후불량적표지;대우PLCH환자응주의PH적조기평고여예방.
Objective To study the clinical features of pulmonary hypertension (PH) complicating pulmonary langerhans' cell histioeytosis (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.