医学
结节病
肺动脉高压
肺纤维化
肺
呼吸系统
纤维化
正电子发射断层摄影术
特发性肺纤维化
肺结节病
病理
内科学
放射科
作者
Rohit Gupta,Robert P. Baughman
出处
期刊:Seminars in Respiratory and Critical Care Medicine
[Georg Thieme Verlag KG]
日期:2020-08-10
卷期号:41 (05): 700-715
被引量:20
标识
DOI:10.1055/s-0040-1709495
摘要
Abstract At least 5% of sarcoidosis patients die from their disease, usually from advanced pulmonary sarcoidosis. The three major problems encountered in advanced pulmonary sarcoidosis are pulmonary fibrosis, pulmonary hypertension, and respiratory infections. Pulmonary fibrosis is the result of chronic inflammation, but other factors including abnormal wound healing may be important. Sarcoidosis-associated pulmonary hypertension (SAPH) is multifactorial including parenchymal fibrosis, vascular granulomas, and hypoxia. Respiratory infections can be cause by structural changes in the lung and impaired immunity due to sarcoidosis or therapy. Anti-inflammatory therapy alone is not effective in most forms of advanced pulmonary sarcoidosis. New techniques, including high-resolution computer tomography and 18F-fluorodeoxyglucose positron emission tomography (PET) have proved helpful in identifying the cause of advanced disease and directing specific therapy.
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