Kasukawa R, Nishimaki T, Takagi T, Miyawaki S, Yokohari R, Tsunematsu T
Second Department of Internal Medicine, Fukushima Medical College, Japan.
Clin Rheumatol. 1990 Mar;9(1):56-62. doi: 10.1007/BF02030243.
Clinical features and prognosis of sixty patients with connective tissue disease accompanied by pulmonary hypertension (PH) (26 MCTD, 20 SLE, and 14 PSS) reported retrospectively by multi-institutions were compared. Though the obtained data were incomplete and lacking in uniformity, no significant difference in the clinical features among the three diseases were observed except high incidence of pulmonary fibrosis and low % VC in PSS and PH patients. Statistically significant difference, however, was observed between live and dead patients of three diseases gathered in post sternal pain, pulmonary diastolic murmur, right ventricular hypertrophy on ECG and mean pressure of pulmonary artery. Higher incidence of anti-nRNP antibody was observed in SLE with PH and PSS with PH patients than with the general population. A quicker occurrence of PH and shorter survival time were observed in MCTD patients with PH than in SLE and PSS patients with PH.
回顾性比较了多机构报告的60例结缔组织病合并肺动脉高压（PH）患者（26例混合性结缔组织病、20例系统性红斑狼疮和14例进行性系统性硬化症）的临床特征和预后。尽管所获数据不完整且缺乏一致性，但除进行性系统性硬化症合并PH患者肺纤维化发生率高和肺活量百分比低外，未观察到这三种疾病临床特征的显著差异。然而，在三种疾病的存活和死亡患者之间，在胸骨后疼痛、肺动脉舒张期杂音、心电图显示右心室肥厚以及肺动脉平均压方面观察到统计学显著差异。与普通人群相比，系统性红斑狼疮合并PH和进行性系统性硬化症合并PH患者中抗nRNP抗体的发生率更高。与系统性红斑狼疮和进行性系统性硬化症合并PH患者相比，混合性结缔组织病合并PH患者发生PH更快且生存时间更短。
