Clinical features and imaging findings in pulmonary capillary hemangiomatosis： report of two cases and a pooled analysis

作     者：XIE Wan-mu DAI Hua-ping JIN Mu-lan WANG Zhen YANG Yuan-hua ZHAI Zhen-guo WANG Chen 

作者机构：Department of Respiratory and Critical Care MedicineBeijing Chao-Yang HospitalCapital Medical UniversityBeijing 100020China Department of PathologyBeijing Chao-Yang HospitalCapital Medical UniversityBeijing 100020China Bei)ing Key Laboratory of Respiratory and Pulmonary CirculationBeijing Institute of Respiratory MedicineBeijing 100020China Beijing Hospital of Ministry of HealthBeijing 1007-30China 

出 版 物：《Chinese Medical Journal》 (中华医学杂志（英文版）)

年 卷 期：2012年第125卷第17期

页      面：3069-3073页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

基　　金：This work was supported by a grant from the Major International Joint Research Project of Natural Science Foundation of China (No. 30810103904) 

主　　题：pulmonary capillary hemangiomatosis pulmonary hypertension lung biopsy 

摘      要：Background Pulmonary capillary hemangiomatosis （PCH） is a rare disease and no Chinese case has been reported yet. The disease is often misdiagnosed and its clinical characteristics are incompletely described. The aim of this study was to describe two Chinese cases and to clarify the clinical and radiographic parameters of patients with PCH. Methods Two PCH cases were presented and other cases were searched from the English literature. All available clinical and radiographic data were collected from 62 literature reported PCH cases. A pooled analysis of total 64 cases was made. Results Dyspnea and hemoptysis were the most common clinical symptoms of PCH. Pulmonary hypertension （PH） was found in 78% of the reported cases. PCH typically showed characteristic diffuse or patchy ground-glass opacities （GGOs） and/or multiple ill-defined centrilobular nodules in the computed tomography. Conclusions The diagnosis of PCH requires a high clinical suspicion. However, both clinical presentations and radiographic studies often provide clues to the diagnosis, which may prompt early lung biopsy for a definite diagnosis.