肺毛细血管血管瘤病(PCH)是由肺毛细血管增生引起的病因不明的肺动脉高压(PH)的罕见原因。临床上,PCH见于性别倾向相同的年轻人,很少报道家族性倾向。PCH的主要临床表现是进行性呼吸困难,疲劳,咯血,心悸,以及后来的不可逆肺动脉高压和右心衰竭。特此,我们报告了三例PCH病例,每个病例都有一个独特的介绍,并有一个全面的文献综述,突出病因,临床表现,建立诊断的诊断方式和病理学,目前的治疗选择,和PCH的预后。总之,将PCH定义为PH的根本原因至关重要,因为大多数用于PH的药物在PCH中无效。由于肺水肿的风险增加,应避免使用血管扩张剂。肺的病理检查仍然被认为是最确定的诊断工具,然而它与并发症风险有关。高分辨率计算机断层扫描(HRCT)胸部目前被认为是诊断PH的基础非侵入性方式。到目前为止,没有明确的PCH治疗,排除肺移植,血管生成抑制剂有初步的有希望的结果。PCH预后极差,自诊断之日起中位生存期为3年。
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) of unknown etiology resulting from pulmonary capillary proliferation. Clinically, PCH is seen in young adults with equal sex predilection and rarely reported familial predisposition. PCH\'s main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations, and later irreversible pulmonary hypertension and right-sided heart failure. Hereby, we report three PCH cases, each case presented with a peculiar presentation with a comprehensive literature
review highlighting etiology, clinical presentations, diagnostic modalities and pathology in establishing a diagnosis, current treatment options, and prognosis of PCH. In conclusion, defining PCH as the underlying cause of PH is of utmost importance as most medications used for PH are ineffective in PCH. Vasodilators should be avoided due to the increased risk of pulmonary oedema. Pathological examination of the lung is still considered the most definitive diagnostic tool, yet it is associated with complications risk. High-Resolution Computed Tomography (HRCT) chest is currently considered the cornerstone non-invasive modality for the diagnosis of PH. So far, no definitive treatment of PCH excluding lung transplantation with preliminary promising results with angiogenesis Inhibitors. PCH carries a very poor prognosis with a median survival of 3 years from the time of diagnosis.