Blom-Bülow B, Jonson B, Brauer K
Eur J Respir Dis. 1985 Jan;66(1):1-8.
Pulmonary function was investigated in 24 patients with progressive systemic sclerosis. The lung volumes were measured by a body plethysmograph. Bronchial and parenchymal properties were studied by relating pulmonary resistance and lung volume to pulmonary elastic recoil pressure. Pulmonary mechanics and arterial blood gases were studied during graded exercise on a bicycle ergometer. The most fundamental abnormality was a less compliant lung parenchyma together with lowered TLC, VC and ventilatory capacity. A mild intrinsic bronchial obstruction was found, irrespective of smoking habits. In 7 patients, resistance did not, however, increase towards residual volume as it does in normal subjects. This phenomenon is interpreted as stiff airways withstanding compression. Blood gas values at rest, and during work, were remarkably normal. There was no correlation between fibrosis judged from static lung compliance and from chest roentgenograms.
对24例进行性系统性硬化症患者的肺功能进行了研究。通过体容积描记器测量肺容积。通过将肺阻力和肺容积与肺弹性回缩压相关联来研究支气管和实质特性。在自行车测力计上进行分级运动期间研究肺力学和动脉血气。最根本的异常是肺实质顺应性降低，同时肺总量、肺活量和通气能力降低。无论吸烟习惯如何，均发现有轻度的内在性支气管阻塞。然而，在7例患者中，阻力并不像正常受试者那样随着残气量增加而增加。这种现象被解释为僵硬的气道抵抗压缩。静息和工作时的血气值非常正常。根据静态肺顺应性和胸部X线片判断的纤维化之间没有相关性。
