Targeting vascular disease in systemic sclerosis.
Systemic sclerosis (scleroderma, SSc) is a chronic connective tissue disease of unknown etiology characterized by progressive fibrosis of the skin and a distinctive pattern of internal organ involvement. Excessive fibrosis, vascular injury, autoimmunity and inflammation are permanent features of the disease process leading to irreversible organ damage and significant morbidity and mortality in SSc patients. Recent progress in understanding the pathogenesis of SSc as well as diagnostic and therapeutic advances in medicine have made more effective treatment strategies possible. So far, therapies targeting vascular aspects of SSc have been most successful. This underlines the role of vascular injury in the pathogenesis of the disease and raising hopes of significant improvement in the management of SSc patients. The aim of this review is to summarize recent and potential future treatments of SSc-associated vascular disease.
Kowal-Bielecka
Otylia
O
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Ul. M. Curie-Sklodowskiej 24 A, 15-276 Bialystok, Poland. otylia@amb.edu.pl
D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000818:Animals; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D016232:Endothelins / Q000037:antagonists & inhibitors; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009569:Nitric Oxide / Q000502:physiology; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D044062:Prostaglandins I / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D014652:Vascular Diseases / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology
D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D002121:Calcium Channel Blockers; D016232:Endothelins; D007166:Immunosuppressive Agents; D010726:Phosphodiesterase Inhibitors; D044062:Prostaglandins I; D009569:Nitric Oxide