Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China.
Chin Med J (Engl). 2012 Sep;125(17):3069-73.
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.
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.
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.
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.
肺毛细血管血管瘤病（PCH）是一种罕见疾病，目前尚未有中文病例报道。该疾病常被误诊，其临床特征也尚未完全描述。本研究旨在描述两例中国病例，并阐明 PCH 患者的临床和影像学参数。
呈现了两例 PCH 病例，并从英文文献中检索了其他病例。从 62 篇文献报道的 PCH 病例中收集了所有可用的临床和影像学数据。对总共 64 例病例进行了汇总分析。
呼吸困难和咯血是 PCH 最常见的临床症状。报告的病例中有 78%存在肺动脉高压（PH）。PCH 典型表现为弥漫性或斑片状磨玻璃影（GGO）和/或多发边界不清的小叶中心结节。
PCH 的诊断需要高度的临床怀疑。然而，临床表现和影像学研究通常都为诊断提供线索，这可能促使进行早期肺活检以明确诊断。
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