肺毛细血管瘤病1例及文献回顾

报道1例肺毛细血管瘤病（(pulmonary capillary hemangiomatosis，PCH)，结合本例特点复习国内外文献报道和2008年WHO在Dana Point制定的肺高压临床分类，对该病的诊断、鉴别诊断以及病因和治疗进行介绍，提高各科医生对这一罕见病的认识。本例患者为中年女性，主要临床症状为活动后气促；右心导管检查示中度肺动脉高压；CT肺血管造影（CT pulmonary angiography, CTPA）示双肺弥漫磨玻璃密度小叶核心结节，肺动脉主干及分支增宽；组织病理学上表现为肺小动脉肌化，肌性肺动脉中膜增厚，肺泡壁毛细血管显著增生呈多排、结节状及片状；患者药物治疗无效，于3个月后死亡。PCH是一种罕见、预后不良的血管病变，其诊断需要综合临床、影像及组织病理3方面的资料，组织病理学检查是最可靠的手段，对确诊具有决定性作用。该病在临床、影像及组织形态上均与肺静脉阻塞病（pulmonary veno occlusive disease, PVOD）存在诸多相似和交叉之处，两者需要重点鉴别。在诸多病理学改变中，肺泡壁毛细血管增生是PCH最重要的特点，也是与PVOD相鉴别的关键点。肺移植被认为是PCH唯一有效的治疗手段，如果不及时进行，患者通常在确诊后几个月内死亡。

Pulmonary capillary hemangiomatosis:a case report and literature review

We reported a case of pulmonary capillary hemangiomatosis (PCH) and introduced its diag-nosis, differential diagnosis, pathogenesis and development of treatment based on the review of Dana Point 2008 Classification of Pulmonary Hypertensiona and current literatures .A 43-year-old female presented progressive dyspnea, elevated pulmonary arterial pressures and CT pulmonary angiography (CTPA) imaging of main pulmonary arterial enlargement and wide spread ill -defined centrilobular nodules of ground-glass opacity.Her histologic features were proliferation of capillary channels within alveolar walls as well as muscularization of arterioles and medial hypertrophy of muscular pulmonary arteries.The treatment with diuretics and warfarin was used promptly , but unfortunately was ineffective. The patient died three months after diagnosis .PCH is a very rare vascular disease with poor prognosis . The diagnosis of PCH rests on the integration of clinical and radiographic information with pathologic fea -tures, however pathology is the most reliable means .Because clinical symptoms, imaging and histological features of pulmonary veno-occlusive disease (PVOD) and PCH broadly overlap, differential diagnosis should be made carefully.Among the various pathologic features proliferation of capillaries within alveolar walls is the key point for diagnosing PCH , which is also the most critical criteria for differentiating PCH from PVOD.So far the only definitive treatment for PCH is lung transplantation , without which the pa-tient will die several months after diagnosis .