Gibson G J, Pride N B
Am Rev Respir Dis. 1977 Oct;116(4):637-47. doi: 10.1164/arrd.1977.116.4.637.
Pulmonary and airway mechanics were studied in 8 patients with severe fibrosing alveolitis. In this disease, the number of functioning alveoli is probably considerably decreased, but static compliance was low even when related to the decreased lung volume. Lung recoil pressures toward full inflation were high but were not increased at and below functional residual capacity. Airway function was broadly normal for lung recoil pressure but supernormal for absolute lung volume. Analysis of model pressure-volume and maximal flow-volume curves showed that (1) these features could be produced simply by replacement of some units by indistensible fibrous tissue and retention of normal function of the surviving alveoli, respiratory muscles, chest wall, and airways; (2) increased lung tissue volume may prevent an increase in lung recoil pressures in the lower part of the vital capacity; and (3) the decreased lung volume could result in a decrease in upstream conductance without structural change in the airways. Measurement of lung compliance, even when corrected for the decreased lung volumes, may not distinguish between loss of units (lung "shrinkage") and abnormal distensibility of the functioning alveoli, but the latter is likely if maximal expiratory flow is excessive in relation to the lung volume expressed as per cent vital capacity.
对8例重症肺纤维化患者的肺和气道力学进行了研究。在这种疾病中，功能正常的肺泡数量可能大幅减少，但即使与减少的肺容积相关，静态顺应性仍较低。朝向完全膨胀的肺回缩压较高，但在功能残气量及以下时并未升高。对于肺回缩压，气道功能大致正常，但对于绝对肺容积则超常。对模型压力-容积曲线和最大流量-容积曲线的分析表明：（1）这些特征可简单地通过用不可扩张的纤维组织替代一些单位，并保留存活肺泡、呼吸肌、胸壁和气道的正常功能来产生；（2）肺组织容积增加可能会阻止肺活量下部肺回缩压的升高；（3）肺容积减小可能导致气道上游传导性降低，而气道结构无变化。即使校正了减少的肺容积，肺顺应性的测量也可能无法区分单位丧失（肺“萎缩”）和功能正常肺泡的异常扩张性，但如果最大呼气流量相对于以肺活量百分比表示的肺容积过高，则可能存在后者情况。
