Pulmonary function testing in idiopathic interstitial pneumonias.
Pulmonary function testing in idiopathic interstitial pneumonias.
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DOI：
10.1513/pats.200602-022tk
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发表时间：
2006-06-01
期刊：
影响因子：
--
通讯作者：
Flaherty, Kevin
中科院分区：
文献类型：
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作者：
Martinez, Fernando J;Flaherty, Kevin
弥漫性实质性肺疾病是一组涉及上皮和内皮基底膜之间的空间的疾病，通常分为四大类。这些包括特发性间质性肺炎，进一步分为7个临床/放射学/病理学亚组。这些疾病通常具有共同的生理异常模式，其特征在于限制性呼吸缺陷和弥散能力降低（DLCO）。肺功能测试经常被用于评估和管理中。生理测试的潜在临床应用包括辅助诊断，尽管其在鉴别诊断中的价值有限。肺功能测试还有助于确定疾病的严重程度和确定预后。在非特异性间质性肺炎和特发性肺纤维化中，DLCO严重降低已被证明在这方面有价值。类似地，基线检测时劳力性去饱和度低于88%和短期随访过程中FVC下降（大于10%）确定患者具有特定的死亡风险。最后，生理测试，特别是肺功能测定和DLCO，已证明在监测治疗反应和确定疾病进展方面的价值。
Diffuse parenchymal lung diseases are a group of disorders that involve the space between the epithelial and endothelial basement membranes and are generally segregated into four major categories. These include the idiopathic interstitial pneumonias, which are further categorized into seven clinical/radiologic/pathologic subsets. These disorders generally share a common pattern of physiologic abnormality characterized by a restrictive ventilatory defect and reduced diffusing capacity (DLCO). Pulmonary function testing is often used and recommended in their assessment and management. The potential clinical application of physiologic testing includes to aid in diagnosis, although its value in differential diagnosis is limited. Pulmonary function testing also aids in establishing disease severity and in defining prognosis. In nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis, severely decreased DLCO has proven valuable in this regard. Similarly, exertional desaturation to less than 88% at baseline testing and a decrease in FVC (greater than 10%) over the course of short-term follow-up identify patients at particular risk of mortality. Finally, physiologic testing, especially spirometry and DLCO, have demonstrated value in monitoring response to therapy and identifying disease progression.