The clinical and immunologic features of pulmonary fibrosis in sarcoidosis.
The clinical and immunologic features of pulmonary fibrosis in sarcoidosis.
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DOI：
10.1016/j.trsl.2012.03.005
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发表时间：
2012-11
期刊：
影响因子：
--
通讯作者：
Niewold TB
中科院分区：
文献类型：
--
作者：
Patterson KC;Hogarth K;Husain AN;Sperling AI;Niewold TB
Sarcoidosis is a multisystem, granulomatous disease that most often affects the lungs. The clinical course is highly variable; many patients undergo spontaneous remission, but up to a third of patients progresses to a chronic disease course. The development of pulmonary fibrosis (PF) in a subset of patients with chronic disease has a negative impact on morbidity and mortality. While sarcoidosis-associated PF can be progressive, it is often referred to as “burnt out” disease, a designation reflecting inactive granulomatous inflammation. The immune mechanisms of sarcoidosis-associated PF are not well understood. It is not clear if fibrotic processes are active from the onset of sarcoidosis in predisposed individuals, or whether a profibrotic state develops as a response to ongoing inflammation. Transforming growth factor β (TGF-β) is an important profibrotic cytokine, and in sarcoidosis, distinct genotypes of TGF-β have been identified in those with PF. The overall cytokine profile in sarcoidosis-associated PF has not been well characterized, although a transition from a T helper 1 to a T helper 2 signature has been proposed. Macrophages have important regulatory interactions with fibroblasts, and the role of alveolar macrophages in sarcoidosis-associated PF is a compelling target for further study. Elucidating the natural history of sarcoidosis-associated PF will inform our understanding of the fundamental derangements, and will enhance prognostication and the development of therapeutic strategies.
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影响因子：
120.7
作者：
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通讯作者：
Fontana, Joseph R.
DOI：
10.1164/ajrccm/144.5.1080
发表时间：
1991-11-01
期刊：
AMERICAN REVIEW OF RESPIRATORY DISEASE
影响因子：
--
作者：
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通讯作者：
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影响因子：
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作者：
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通讯作者：
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影响因子：
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作者：
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通讯作者：
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DOI：
10.1165/rcmb.2011-0025rt
发表时间：
2011-09-01
影响因子：
6.4
作者：
Bozyk, Paul D.;Moore, Bethany B.
通讯作者：
Moore, Bethany B.