Pronk L C, Swaak A J
Department of Internal Medicine, Dr Daniel den Hoed Clinic, Rotterdam, The Netherlands.
Rheumatol Int. 1991;11(2):83-6. doi: 10.1007/BF00291151.
Patients with connective tissue disease (CTD) who are prone to developed isolated pulmonary hypertension (PH) are primarily young females with a history of Raynaud's phenomenon associated with an exertional dyspnoea. From the start of the disease, pulmonary function tests show a decreased diffusing capacity for carbon monoxide, while X-ray examination shows no obvious abnormalities such as interstitial fibrosis. All patients show, on electrocardiographic examination, evidence of right axis deviation and right ventricular hypertrophy. It has been suggested that PH is found mostly in patients with systemic scerlosis characterized by the CREST syndrome. The histopathological findings are intimal proliferation, narrowing of the vessel lumen and medial fibrosis. These are not specific for CTD. One would expect more signs of vasculitis. Neither signs of lung fibrosis, nor signs of pulmonary emboli are described.
易患孤立性肺动脉高压（PH）的结缔组织病（CTD）患者主要是年轻女性，有雷诺现象病史并伴有劳力性呼吸困难。从疾病开始，肺功能测试显示一氧化碳弥散能力下降，而X线检查无明显异常，如间质纤维化。所有患者心电图检查均显示电轴右偏和右心室肥厚。有人认为PH多见于以CREST综合征为特征的系统性硬化症患者。组织病理学表现为内膜增生、血管腔狭窄和中层纤维化。这些并非CTD所特有。人们预期会有更多血管炎的迹象。文中未描述肺纤维化迹象或肺栓塞迹象。
