Research Unit for Molecular Lung and Pleura Pathology, Institute of Pathology, Medical University of Graz, Auenbruggerplatz 25, Graz, 8036, Austria.
Virchows Arch. 2013 Jan;462(1):1-26. doi: 10.1007/s00428-012-1305-0. Epub 2012 Dec 7.
Interstitial lung diseases (ILD) encompass a group of diseases with a wide range of etiologies and a variety of tissue reactions within the lung. In many instances, a careful evaluation of the tissue reactions will result in a specific diagnosis or at least in a narrow range of differentials, which will assist the clinician to arrive at a definite diagnosis, when combining our interpretation with the clinical presentation of the patient and high-resolution computed tomography. In this review, we will exclude granulomatous pneumonias as well as vascular diseases (primary arterial pulmonary hypertension and vasculitis); however, pulmonary hypertension as a complication of interstitial processes will be mentioned. Few entities of pneumoconiosis presenting as an interstitial process will be included, whereas those with granulomatous reactions will be excluded. Drug reactions will be touched on within interstitial pneumonias, but will not be a major focus. In contrast to the present-day preferred descriptive pattern recognition, it is the author's strong belief that pathologists should always try to dig out the etiology from a tissue specimen and not being satisfied with just a pattern description. It is the difference of sorting tissue reactions into boxes by their main pattern, without recognizing minor or minute reactions, which sometimes will guide one to the correct etiology-oriented interpretation. In the author's personal perspective, tissue reactions can even be sorted by their timeliness, and therefore, ordered by the time of appearance, providing an insight into the pathogenesis and course of a disease. Also, underlying immune mechanisms will be discussed briefly as far as they are essential to understand the disease.
间质性肺疾病（ILD）包含一组具有广泛病因和多种肺部组织反应的疾病。在许多情况下，对组织反应的仔细评估将导致特定的诊断，或者至少可以缩小鉴别诊断的范围，这将有助于临床医生在结合患者的临床表现和高分辨率计算机断层扫描结果进行综合分析后做出明确的诊断。在本篇综述中，我们将排除肉芽肿性肺炎和血管疾病（原发性肺动脉高压和血管炎）；然而，作为间质性病变并发症的肺动脉高压将会提及。少数表现为间质性病变的尘肺将会包含在内，而那些有肉芽肿性反应的则将排除在外。药物反应将在间质性肺炎中提及，但不会是重点。与当前首选的描述性模式识别不同，作者强烈认为病理学家应该始终尝试从组织标本中挖掘病因，而不仅仅满足于模式描述。正是将组织反应按主要模式分类而不识别次要或微小反应的做法，有时会引导我们做出正确的病因导向的解释。从作者的个人角度来看，甚至可以根据组织反应的时效性进行分类，因此，可以根据出现的时间顺序进行排序，从而深入了解疾病的发病机制和病程。此外，还将简要讨论潜在的免疫机制，因为理解疾病的发病机制和病程需要了解免疫机制。
