关键词: 肺毛细血管瘤病, 肺静脉闭塞性疾病, 肺动脉高压

Abstract:

SUMMARYWe reported a case of pulmonary capillary hemangiomatosis (PCH) and introduced its diagnosis, 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 features, 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 patient will die several months after diagnosis.

Key words: Pulmonary capillary hemangiomatosis, Pulmonary veno-occlusive disease, Pulmonary arterial hypertension