Mousapasandi Amir, Herbert Cristan, Thomas Paul
School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.
School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia
J Investig Med. 2021 Jan 15. doi: 10.1136/jim-2020-001659.
Sarcoidosis is a systemic granulomatous disease of unknown etiology and pathogenesis with a heterogeneous clinical presentation. In the appropriate clinical and radiological context and with the exclusion of other diagnoses, the disease is characterized by the pathological presence of non-caseating epithelioid cell granulomas. Sarcoidosis is postulated to be a multifactorial disease caused by chronic antigenic stimulation. The immunopathogenesis of sarcoidosis encompasses a complex interaction between the host, genetic factors and postulated environmental and infectious triggers, which result in granuloma development.The exact pathogenesis of the disease has yet to be elucidated, but some of the inflammatory pathways that play a key role in disease progression and outcomes are becoming apparent, and these may form the logical basis for selecting potential biomarkers.Biomarkers are biological molecules that are altered pathologically. To date, there exists no single reliable biomarker for the evaluation of sarcoidosis, either diagnostically or prognostically but new candidates are emerging. A diagnosis of sarcoidosis ideally requires a biopsy confirming non-caseating granulomas, but the likelihood of progression that requires intervention remains unpredictable. These challenging aspects could be potentially resolved by incorporating biomarkers into clinical practice for both diagnosis and monitoring disease activity.This review outlines the current knowledge on sarcoidosis with an emphasis on pulmonary sarcoidosis, and delineates the understanding surrounding the implication of biomarkers for the clinical evaluation of sarcoidosis.
结节病是一种病因和发病机制不明的全身性肉芽肿性疾病,临床表现多样。在合适的临床和影像学背景下,排除其他诊断后,该病的特征是存在非干酪样上皮样细胞肉芽肿。结节病被认为是一种由慢性抗原刺激引起的多因素疾病。结节病的免疫发病机制包括宿主、遗传因素与假定的环境及感染触发因素之间的复杂相互作用,这些相互作用导致肉芽肿的形成。该病的确切发病机制尚待阐明,但一些在疾病进展和转归中起关键作用的炎症途径正逐渐显现,这些可能构成选择潜在生物标志物的逻辑基础。生物标志物是发生病理改变的生物分子。迄今为止,无论是在诊断还是预后评估方面,都不存在单一可靠的用于评估结节病的生物标志物,但新的候选标志物正在不断涌现。结节病的诊断理想情况下需要活检证实非干酪样肉芽肿,但需要干预的疾病进展可能性仍然不可预测。通过将生物标志物纳入临床实践用于诊断和监测疾病活动,这些具有挑战性的方面可能会得到潜在解决。本综述概述了目前关于结节病的知识,重点是肺结节病,并阐述了围绕生物标志物对结节病临床评估意义的理解。