Pulmonary hypertension associated with sarcoidosis.
Pulmonary hypertension associated with sarcoidosis.
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DOI：
10.1186/ar2192
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发表时间：
2007
影响因子：
4.9
通讯作者：
Baughman RP
中科院分区：
文献类型：
--
作者：
Baughman RP
肺受累在结节病中很常见，结节病是一种免疫介导的炎症性疾病，其特征是组织中的非干酪化肉芽肿。患有晚期肺部疾病，特别是终末期肺纤维化的结节病患者，有发生肺动脉高压的风险（世界卫生组织III组继发于缺氧性肺病的肺动脉高压）。某些结节病患者血浆和支气管肺泡灌洗液中内皮素（ET）-1水平升高，提示ET-1可能是肺纤维化和结节病相关性肺动脉高压的驱动因素。虽然ET-1水平升高与临床表型之间的关系尚未确定，但来自波生坦等药物阻断ET-1研究的早期证据令人鼓舞。这种治疗可能与标准抗炎药联合使用以改善预后。
Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organization group III pulmonary hypertension secondary to hypoxic lung disease). Increased levels of endothelin (ET)-1 in plasma and bronchoalveolar lavage of some sarcoid patients suggest that ET-1 may be driving pulmonary fibrosis and sarcoidosis-associated pulmonary hypertension. Although a relationship between raised levels of ET-1 and clinical phenotype is yet to be identified, early evidence from studies of ET-1 blockade with drugs such as bosentan is encouraging. Such therapy possibly could be combined with standard anti-inflammatory agents to improve outcome.
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影响因子：
5.7
作者：
Doughan, AR;Williams, BR
通讯作者：
Williams, BR
影响因子：
9.6
作者：
Arcasoy, SM;Christie, JD;Kotloff, RM
通讯作者：
Kotloff, RM
影响因子：
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作者：
Eishi, Y;Suga, M;Ando, M
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作者：
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通讯作者：
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DOI：
10.1164/rccm.200603-402oc
发表时间：
2006-10-01
影响因子：
24.7
作者：
Baughman, Robert P.;Drent, Marjolein;Barnathan, Elliot S.
通讯作者：
Barnathan, Elliot S.