From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis.
From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis.
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DOI：
10.1097/mcp.0000000000000301
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发表时间：
2016-09
影响因子：
3.3
通讯作者：
Patterson KC
中科院分区：
文献类型：
--
作者：
Bonham CA;Strek ME;Patterson KC
高达20%的结节病患者发展为肺纤维化，将通常良性的疾病转变为高度病态和潜在致命的疾病。我们强调纤维化性肺结节病表型是一个强烈的临床和转化研究领域，回顾了最近的治疗进展，并为结节病相关肺纤维化的未来研究提供了路线图。淋巴分布的肉芽肿性炎症是肺结节病和纤维化病灶的标志性发现。最近的研究表明，纤维化结节病始于持续的、不受控制的炎症，并与促纤维化的遗传特征和免疫反应有关。与其他纤维化肺部疾病的比较也揭示了关键特征，使我们了解纤维化的共同途径。了解结节病纤维化转化的机制可以提高临床护理水平，促进新的治疗方案的发展。这些发现对纤维化结节病的影响可能通过应用于其他以炎症向纤维化转化为特征的间质性肺疾病而被放大。纤维化结节病临床管理的重要方面包括监测合并症，如肺动脉高压、气道疾病和感染，以及评估可能受益于免疫抑制的肺部疾病活动性。
Up to twenty percent of patients with sarcoidosis develop pulmonary fibrosis, transforming an often benign disease into a highly morbid and potentially fatal one. We highlight the fibrotic pulmonary sarcoidosis phenotype as an area of intense clinical and translational investigation, review recent developments in treatment, and provide a roadmap for future research in sarcoidosis associated pulmonary fibrosis. Granulomatous inflammation in a lymphatic distribution is the hallmark finding of pulmonary sarcoidosis and the nidus for fibrosis. Recent research demonstrates that fibrotic sarcoidosis begins in the setting of persistent, uncontrolled inflammation, and is aided by pro-fibrotic genetic features and immune responses. Comparison to other fibrotic lung diseases also reveals key features that inform our understanding of common pathways in fibrosis. Understanding the mechanisms of fibrotic transformation in sarcoidosis enhances clinical care and facilitates development of novel therapeutic options. The impact of these findings in fibrotic sarcoidosis may be amplified through application to other interstitial lung diseases marked by inflammatory to fibrotic transformation. Important aspects of clinical management of fibrotic sarcoidosis include surveillance for co-morbidities, such as pulmonary hypertension, airway disease, and infection, and assessment for pulmonary disease activity that may benefit from immunosuppression.