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10.1016/j.rdc.2014.12.005

http://scihub22266oqcxt.onion/10.1016/j.rdc.2014.12.005
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C4385198!4385198!25836640
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suck abstract from ncbi


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pmid25836640      Rheum+Dis+Clin+North+Am 2015 ; 41 (2): 237-48
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  • Interstitial Lung Disease in Scleroderma #MMPMID25836640
  • Schoenfeld SR; Castelino FV
  • Rheum Dis Clin North Am 2015[May]; 41 (2): 237-48 PMID25836640show ga
  • Systemic sclerosis (SSc) is a heterogeneous disease of unknown etiology and with limited effective therapies. It is characterized by autoimmunity, vasculopathy and fibrosis and is clinically manifested by multi-organ involvement. Interstitial lung disease (ILD) is a common complication of the disease and is associated with significant morbidity and mortality. The diagnosis of ILD hinges upon careful clinical evaluation as well as pulmonary function tests (PFTs) and high resolution computed tomography (HRCT). A number of pro-inflammatory and pro-fibrotic mediators are involved in the pathogenesis of SSc-ILD, with transforming growth factor-beta (TGF-?) playing a key role in the development of fibrosis. Despite recent advances in the understanding of the mechanisms of disease initiation and progression, effective therapeutic options are still limited. A number of experimental therapies are currently in early phase clinical trials and show promise.
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