Mădălina Matei, Cristian Constantin Budacu , Gabi Topor


Scleroderma (systemic sclerosis) is a rare, progressive, autoimmune chronic disease that affects approximately 2.5 million people worldwide, characterized by diffuse fibrosis and vascular abnormalities in the skin, joints and internal organs (especially esophagus, lower gastrointestinal tract, lungs, heart, kidneys). In immunological smelting mechanisms and heredity, certain HLA subtypes are involved that play a role in the etiology of the disease. The pathophysiological mechanisms consist of vascular lesions with the activation of fibroblasts; excessive synthesis of collagen and other extracellular proteins occurs in different tissues. The disease affects four times more frequently women than men, and is more common in the age group 20-50 years.Genetic investigations have focused on candidate genepolymorphisms, particularly those involved in regulating  immunity and inflammation, vascular function and connective tissue homeostatis Material and method: Scleroderma is a disease with complex pathogenesis (vascular, immune and extended fibrosis) and severe organic disorders (skin, lung, kidney, heart, gastrointestinal tract) which requires the application of therapeutic measures as early as possible. The study includes 11 patients with systemic scleroderma.Results and discussions: Medication therapy consists mainly of the treatment aimed at the complications of the disease and the assurance of symptomatic relief. This includes: vasodilating agents (calcium channel blockers, angiotensin converting enzyme inhibitors, topical nitroglycerin, prostaglandin analogues, endothelin receptor inhibitors, 5 phosphodiesterase inhibitors); immunosuppressive / antifibrotic agents (Cyclophosphamide, Methotrexate, Cyclosporine, anti – TGFβ).  

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