Bokulic R E, Hilman B C
Department of Pediatrics, Children's Hospital Medical Center, Cincinnati, Ohio.
Pediatr Clin North Am. 1994 Jun;41(3):543-67. doi: 10.1016/s0031-3955(16)38769-7.
Interstitial lung disease in children is a heterogeneous group of disorders of both known and unknown causes that share a common histologic characteristic (i.e., inflammation of the pulmonary interstitium that may resolve completely, partially, or progress to derangement of alveolar structures with varying degrees of fibrosis). The inflammatory process, evoked as a result of injury to alveolar epithelium and/or the endothelium, is responsible for alveolar wall thickening that is the histologic marker of ILD. This article extrapolates some of the known pathogenic mechanisms of ILD from adult and animal models and applies this information for a better understanding of the pathogenesis of ILD in children. The clinical manifestations vary and are often subtle and nonspecific. There is no consensus on specific criteria for the clinical diagnosis of ILD in children. There are no pathognomonic laboratory criteria for the diagnosis of ILD in children other than the characteristic findings on histologic examination of the lung. It is important to make the diagnosis early to minimize lung damage. Therapy is directed toward the reduction of the inflammatory response to minimize or prevent the progression to fibrosis. ILD suffers from lack of uniform guidelines for diagnostic evaluation, therapy, and prognostic indicators essential for critical monitoring of disease activity. No one medical center has enough cases of ILD in children to allow objective evaluation of a significant number of cases with adequate longitudinal follow-up to determine guidelines for optimal management and to identify accurate prognostic indicators. The organization of a multicenter approach will guide us towards a better understanding of ILD in children.
儿童间质性肺疾病是一组病因已知和未知的异质性疾病，它们具有共同的组织学特征（即肺间质炎症，可完全缓解、部分缓解或进展为肺泡结构紊乱并伴有不同程度的纤维化）。炎症过程是由于肺泡上皮和/或内皮损伤引起的，它导致肺泡壁增厚，这是ILD的组织学标志。本文从成人和动物模型中推断出一些已知的ILD致病机制，并应用这些信息来更好地理解儿童ILD的发病机制。其临床表现各异，通常较为隐匿且不具特异性。儿童ILD的临床诊断尚无统一的具体标准。除了肺部组织学检查的特征性发现外，儿童ILD的诊断没有特异性的实验室标准。早期诊断很重要，以便将肺损伤降至最低。治疗旨在减轻炎症反应，以尽量减少或防止进展为纤维化。ILD缺乏关于诊断评估、治疗和预后指标的统一指南，而这些指标对于密切监测疾病活动至关重要。没有一个医疗中心有足够多的儿童ILD病例来对大量病例进行客观评估，并进行充分的纵向随访，以确定最佳管理指南并识别准确的预后指标。多中心研究方法将有助于我们更好地了解儿童ILD。
