title: Micro- and macrovascular treatment targets in scleroderma heart disease.
abstract: Cardiac involvement in systemic sclerosis (SSc) is a frequent visceral complication that considerably affects the prognosis of the disease. The pathophysiologic hallmark is myocardial fibrosis which can progress leading to arrhythmia, right and/or left heart dysfunction and failure. Symptoms range from unusual to prominent and from mild to dramatic, but clinically overt disease is a poor prognostic factor. Primary myocardial involvement is related to focal ischemia due to transient coronary spasm, and the available data support that microvascular functional and structural abnormalities rather than macrovascular coronary involvement represent the main underlying mechanism of the disease. However, the existence and prevalence of atherosclerotic coronary artery disease in SSc remain to be determined, as several studies have generated conflicting reports. Despite the lack of effective targeted therapy for SSc itself, sensitive and quantitative techniques have demonstrated the ability of vasodilators to improve myocardial function and perfusion and to prevent the evolution of subclinical heart involvement to decompensated heart failure. Further research will provide a better understanding of the disease by detecting the potent contribution of coronary artery involvement, explaining differences in accelerated atherosclerosis between SSc and other autoimmune disorders, and opening directions for the development of novel treatment strategies for this life-threatening complication of SSc.
authors.forename: Theodoros
authors.initials: T
authors.lastname: Dimitroulas
authors.forename: George
authors.initials: G
authors.lastname: Giannakoulas
authors.forename: Haralambos
authors.initials: H
authors.lastname: Karvounis
authors.forename: Alexandros
authors.initials: A
authors.lastname: Garyfallos
authors.forename: Lucas
authors.initials: L
authors.lastname: Settas
authors.affiliation: Department of Rheumatology, Dudley Group NHS FT, Russells Hall Hospital, Dudley, DY1 2HQ, UK. dimitroul@hotmail.com.
authors.forename: George D
authors.initials: GD
authors.lastname: Kitas
mesh_terms: D002316:Cardiotonic Agents / Q000627:therapeutic use*; D003331:Coronary Vessels / Q000187:drug effects*; D006321:Heart / Q000187:drug effects*; D006331:Heart Diseases / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D055806:Microvessels / Q000187:drug effects*; D058990:Molecular Targeted Therapy; D012595:Scleroderma, Systemic / Q000503:physiopathology*; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D002316:Cardiotonic Agents; D014665:Vasodilator Agents