[Fibrotic diseases]

Abstract

Fibrosis is a pathologic process, which includes scar formation and over production of extracellular matrix, by the connective tissue, as a response to tissue damage. The molecular process is not different from normal formation of connective tissue and extracellular matrix in the normal organs. The context, the environment and the over production make the difference. Fibrosis formation includes interaction between many cell types and cytokines, and when the balance becomes profibrotic, there is fibrosis formation. Major profibrotic agents are type 2 CD4 positive lymphocytes, CD40 receptor and ligand interaction, and the following cytokines: IL-4, transforming growth factor b, platelet derived growth factor. The major antifibrotic agent is interferon gamma. Pathologies include: in the skin pathologic scarring as colloid and hypertrophic scar, cirrhosis of liver and gallbladder, in the heart and the kidneys, pulmonary and bone-marrow fibrosis, and scleroderma. Scleroderma is chronic connective tissue disease, expressed clinically by systemic sclerosis and diffuses fibrosis of the skin and viscera. This is a progressive degenerative disorder of the blood vessels, skin, lungs, kidneys, heart and GI tract and for this reason this disease plays a major role in fibrosis research. Fibrosis is considered an irreversible process, at least clinically, and is usually treated by anti-inflammatory and immunosuppressive agents. This kind of therapy was not proven successful and sometimes it harms more than cures. Many patients suffer from fibrotic diseases and the aim is to develop anti-fibrotic agents, targeted to the pathologic molecular process. Progressing step by step in this issue has direct clinic affect.