Bagg L R, Hughes D T
Thorax. 1979 Apr;34(2):224-8. doi: 10.1136/thx.34.2.224.
Serial pulmonary function tests were performed on nine patients with progressive systemic sclerosis over a mean period of ten years. Abnormality was seen to develop both early and late in the course of the disease, and the earliest abnormality observed was impairment of the transfer factor. Deterioration of some aspect of pulmonary function was noted in each case, evidence of restriction or air trapping being seen with equal frequency. Two patients died, both of causes unrelated to their pulmonary involvement, and even pronounced early involvement of the lungs did not necessarily imply a bad prognosis.
对9例进行性系统性硬化症患者进行了平均为期10年的系列肺功能测试。在疾病过程的早期和晚期均可见异常，观察到的最早异常是转运因子受损。在每个病例中均注意到肺功能某些方面的恶化，限制性或气体潴留的证据出现频率相同。2例患者死亡，均死于与肺部受累无关的原因，即使肺部早期明显受累也不一定意味着预后不良。
