Stupi A M, Steen V D, Owens G R, Barnes E L, Rodnan G P, Medsger T A
Arthritis Rheum. 1986 Apr;29(4):515-24. doi: 10.1002/art.1780290409.
Pulmonary hypertension (PHT) occurred in 59 (9%) of 673 systemic sclerosis patients seen between 1963 and 1983. In 30 patients, all with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), the pulmonary hypertension was isolated, i.e., independent of other pulmonary or cardiac conditions. In 20 patients, isolated PHT was demonstrated by cardiac catheterization. All had normal or only mildly decreased lung volumes, and mild or no pulmonary interstitial fibrosis on chest roentgenogram. In comparison with 287 CREST syndrome patients without PHT, these 20 patients had markedly reduced diffusing capacity for carbon monoxide (DLCO) (mean 39% of predicted normal). In 6 patients, the low DLCO antedated clinical evidence of PHT by 1-6 years. At autopsy there was marked intimal fibrosis with hyalinization and smooth muscle hypertrophy in the small- and medium-sized arteries, without significant parenchymal fibrosis or inflammation. Patients with isolated PHT did not respond favorably to vasodilators and had a very poor prognosis, with a 2-year cumulative survival rate of 40%. A DLCO less than 45% of predicted in the absence of pulmonary interstitial fibrosis may be an important predictor of the subsequent development of isolated PHT.
1963年至1983年间诊治的673例系统性硬化症患者中，59例（9%）发生了肺动脉高压（PHT）。在30例患者中，均患有CREST综合征（钙质沉着、雷诺现象、食管动力障碍、指端硬化、毛细血管扩张），其肺动脉高压是孤立存在的，即独立于其他肺部或心脏疾病。20例患者经心导管检查证实为孤立性PHT。所有患者肺容量正常或仅轻度降低，胸部X线片显示轻度或无肺间质纤维化。与287例无PHT的CREST综合征患者相比，这20例患者的一氧化碳弥散量（DLCO）显著降低（平均为预测正常值的39%）。6例患者在出现PHT临床证据前1至6年DLCO就已降低。尸检发现中小动脉有明显的内膜纤维化伴玻璃样变和平滑肌肥大，无明显的实质纤维化或炎症。孤立性PHT患者对血管扩张剂反应不佳，预后很差，2年累积生存率为40%。在无肺间质纤维化的情况下，DLCO低于预测值的45%可能是孤立性PHT后续发生的重要预测指标。
