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contentuptodate· Content· item f31_54_32619

©2013 UpToDate ® Print Email Antifibrotic therapies for systemic sclerosis Treatment Mechanism of action Comments D-penicillamine Inhibits the formation of stable collagen cross-links by forming hydroxy-lysine aldehyde and lysine groups on collagen precursors. Widely used in UK, doses above 750 mg daily probably needed for any benefit. Open studies have shown beneficial effects on lung, skin and kidney disease in SSc. Placebo controlled trial currently underway in USA. Interferon alpha Inhibits collagen production by dermal SSc fibroblasts in vitro, at transcriptional level. May also eliminate high collagen producing fibroblast subpopulations. Efficacy has been confirmed in open studies and a placebo controlled trial is currently underway in UK. Benefits must be balanced by the considerable morbidity of IFN administration. Interferon gamma Similar to interferon alpha. Initial support for use from open studies and in vitro properties, but subsequent evaluation has been less favorable. Colchicine Inhibition of collagen production by disrupting microtubule formation in fibroblast cytoskeleton. Possible enhancement of collagenase activity. Little used in most centers.