Is There Evidence for Vasculitis in Systemic Sclerosis?
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Systemic sclerosis (SSc) is a devastating and potentially life-threatening multi-organ system disease. SSc is marked by skin thickening and tightening, Raynaud’s phenomenon and digital ischemia with ulceration, gastrointestinal dysmotility, cardiopulmonary involvement with pulmonary fibrosis and pulmonary arterial hypertension, as well as renal failure. Fibrosis is the most obvious manifestation of SSc. Vascular involvement and inflammation are other prominent components of SSc pathology, and both features are also seen in vasculitis. This review analyzes whether there is evidence for vasculitis especially with particular organ manifestations and subgroups of patients.
KeywordsSystemic sclerosis Scleroderma Vasculopathy Vasculitis Angiogenesis Vasculogenesis Endothelial cells ANCA
Dr. Saketkoo has served as a consultant for Gilead Sciences and Questcor Pharmaceuticals and has received grant support from United Therapeutics Corp., InterMune, Boehringer Ingelheim, Actelion Pharmaceuticals Ltd., Celgene Corp., and Sigma-Tau Pharmaceuticals.
Dr. Distler has served as a consultant for Actelion Pharmaceuticals Ltd., Pfizer, Boehringer Ingelheim, Bayer, Roche, Ergonex Pharma, Bristol-Myers Squibb, Sanofi-Aventis, United BioSource Corp., Medac, Biovitrum, Novartis, Active Biotech, 4D Science, and Sinoxa Pharma; has received grant support from Actelion Pharmaceuticals Ltd., Pfizer, Ergonex Pharma, and Sanofi-Aventis; has had travel/accommodations expenses covered/reimbursed by Roche, Actelion Pharmaceuticals Ltd., and Pfizer; and holds a patent on miR-29 for the treatment of systemic sclerosis.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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