title: The Pathogenesis of Systemic Sclerosis.
abstract: Systemic sclerosis is a multisystem disorder with a high associated mortality. The hallmark abnormalities of the disease are in the immune system, vasculature, and connective tissue. Systemic sclerosis occurs in susceptible individuals and is stimulated by initiating events that are poorly understood at present. In order for the disease phenotype to appear there is dysfunction in the homoeostatic mechanisms of immune tolerance, endothelial physiology, and extracellular matrix turnover. The progression of disease is not sequential but requires simultaneous dysfunction in these normal regulatory mechanisms. Better understanding of the interplay of these factors is likely to contribute to improved treatment options.
authors.affiliation: Centre for Rheumatology, UCL Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Edward P
authors.initials: EP
authors.lastname: Stern
authors.affiliation: Centre for Rheumatology, UCL Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK. Electronic address: c.denton@ucl.ac.uk.
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Denton
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D018925:Chemokines / Q000502:physiology; D055513:Connective Tissue Growth Factor / Q000502:physiology; D016207:Cytokines / Q000502:physiology; D018450:Disease Progression; D004703:Endocrine System / Q000502:physiology; D005260:Female; D005347:Fibroblasts / Q000502:physiology; D055106:Genome-Wide Association Study; D006680:HLA Antigens / Q000276:immunology; D006801:Humans; D007111:Immunity, Cellular / Q000502:physiology; D007239:Infections / Q000150:complications; D007378:Interleukins / Q000502:physiology; D009369:Neoplasms / Q000150:complications; D011928:Raynaud Disease / Q000150:complications; D012595:Scleroderma, Systemic / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D014662:Vasoconstrictor Agents / Q000494:pharmacology
chemical_list: D001323:Autoantibodies; D018925:Chemokines; D016207:Cytokines; D006680:HLA Antigens; D007378:Interleukins; D014662:Vasoconstrictor Agents; D055513:Connective Tissue Growth Factor
keywords: Autoimmunity; Fibrosis; Pathogenesis; Scleroderma; Systemic sclerosis; Vasculopathy