A perspective view on widespread pulmonary fibrosis.
A perspective view on widespread pulmonary fibrosis.
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对广泛性肺纤维化的透视。
DOI：
10.1136/bmj.2.5915.371
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发表时间：
1974
影响因子：
--
通讯作者：
M. Turner‐Warwick
中科院分区：
文献类型：
--
作者：
M. Turner‐Warwick
广泛的纤维化瘢痕形成是许多不同类型的肺组织损伤愈合的最后阶段。在某些情况下，急性期可能是短暂的;在其他情况下，活跃的过程可能会随着纤维化反应持续很长一段时间。在任何一种情况下，所产生的纤维化都会逐渐损害肺功能，并经常导致过早死亡。因此，乍看起来，当控制和治疗早期过程更为重要时，把重点放在疾病的疤痕组织阶段似乎有点虚无主义。这一主题在简单的临床基础上是合理的。患者经常表现出严重的广泛的肺纤维化，伴有严重的呼吸和循环障碍，在这个阶段，没有任何形式的治疗可以改变他们无情的下坡进程。由于肺部有很大的呼吸储备，因此可能没有或相对轻微的症状，因此，对允许更严重的广泛变化继续下去的危害缺乏认识是可以理解的。即使认识到了潜在的危险，监测最常见的无症状患者疾病进展或消退的最佳方法仍不完全清楚。然而，即使有这种限制，也往往很少或根本没有系统地试图监督这些病人，即使有现成的技术。对静息状态下肺功能的系列评估无论如何都是一个相对粗糙的尺度，而且通常只是在不规则的时间间隔内进行测量;标准化的运动试验很少使用，而且往往过于依赖胸片，胸片也只能反映相对粗略的变化。在任何情况下，胸部X线片都只是阴影，除了少数值得注意的例外，它们可以指示纤维化存在的程度与更急性的可逆性炎性病变不同，是有限的。本讲座将强调各种各样的完全不同的过程，这些过程可能引起肺内广泛的纤维化，将研究其选择性分布的有趣问题--在某些疾病中有利于上叶，而在另一些疾病中有利于下叶--并将考虑影响这些病变进展速度的已知因素。
Widespread fibrotic scarring occurs as a final stage of healing in many different types of tissue damaging injury to the lung. In some cases the acute stage may be short-lived; in others the active process may continue alongside the fibrosing response over prolonged periods. In either event the resulting fibrosis progressively impairs lung performance and often leads to premature death. At first sight it may, therefore, appear to be a somewhat nihilistic exercise to focus upon the scar tissue stage of disease when control and treatment of the earlier processes are of so much greater importance. The topic is justified on simple clinical grounds. Patients are seen regularly presenting with far advanced widespread fibrosis of the lungs with gross venti latory and circulatory impairment, in whom at this stage no form of treatment can alter their relentless downhill progress. The lack of appreciation of the hazards of allowing the more acute widespread changes to continue is understandable be cause, owing to the great ventilatory reserves of the lung, symp toms may be absent or relatively slight. Even when the potential dangers are recognized, the best methods to monitor progress or regression of disease in the aknost asymptomatic patient are not yet fully known. Nevertheless, even with this limitation, all too often little or no systematic attempt is made to supervise these patients, even with the techniques which are readily available. Serial assessment of lung performance at rest is in any case a relatively crude yardstick, and is often measured only at irregular intervals; standardized exercise tests are rarely used, and all too often too much reliance is placed on the chest radiograph, which also reflects only relatively gross changes. In any case chest x-ray films are but shadows, and the extent to which they can indicate the presence of fibrosis as distinct from more acute reversible inflammatory lesions is, with a few notable exceptions, limited. This lecture will emphasize the great variety of entirely distinct processes which may initiate widespread fibrosis in the lungs, will examine the intriguing problem of its selective distribution-favouring characteristically the upper lobes in somediseases and the lower lobes in others-and will consider the known factors which influence the rate of progression of these lesions.