Immunosuppression for interstitial lung disease in systemic sclerosis – novel insights and opportunities for translational research
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Systemic sclerosis (SSc) is a chronic inflammatory disorder characterized by a disturbance in fibroblast function culminating in the telltale skin thickening and fibrosis of visceral organs. Interstitial lung disease (ILD) is common (Steele et al. 2011) and is the leading cause of death in this disease (Steen and Medsger 2007). The immunohistopathogenesis of SSc-ILD is characterized by immune dysfunction and inflammation. Thus, immunosuppression has been hypothesized as a useful treatment for SSc-ILD. However, randomized clinical trials (RCTs) have thus far only revealed a modest effect of immunosuppression (Hoyles et al. 2006; Tashkin et al. 2006). We believe that these small observed effects are due, at least in part, to the actual design of the RCTs, in particular subject selection, which did not properly identify patients likely to respond to treatment.
SSc is an uncommon disease, with an estimated prevalence ranging from 7–489/million and incidence from 0.6–122/million/year...
KeywordsImmunosuppression Interstitial lung disease Outcomes research Systemic sclerosis Translational research
This study was funded in part by the Canadian Institutes of Health Research, the Scleroderma Society of Canada and educational grants from Actelion Pharmaceuticals and Pfizer Inc. Dr. Hudson is supported by a Chercheur-clinicien boursier award from the Fonds de la Recherche en Santé du Québec. The funding sources had no role in the design of the study, analysis of the data, preparation of the manuscript and decision to submit for publication.
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