医学
结节病
自然史
肺纤维化
纤维化
肺移植
肺
肺动脉高压
特发性肺纤维化
肺康复
重症监护医学
内科学
病理
肺病
作者
Hilario Nunès,Pierre‐Yves Brillet,Jean‐François Bernaudin,Thomas Gille,Dominique Valeyre,Florence Jeny
标识
DOI:10.1016/j.ccm.2023.08.011
摘要
Fibrotic pulmonary sarcoidosis (fPS) affects about 20% of patients. fPS carries a significant morbidity and mortality. However, its prognosis is highly variable, depending mainly on fibrosis extent, functional impairment severity, and the development of pulmonary hypertension. Moreover, fPS outcomes are also influenced by several other complications, including acute exacerbations, and infections. fPS natural history is unknown, in particular regarding the risk of progressive self-sustaining fibrosis. The management of fPS is challenging, including anti-inflammatory treatment if granulomatous activity persists, rehabilitation, and in highly selected patients antifibrotic treatment and lung transplantation .
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