Systemic Sclerosis Research Paper

Systemic sclerosis, is a rare connective tissue disease characterized by vascular and immune dysfunction, leading to fibrosis that can damage multiple organs. 4 The clinical forms rang from limited skin involvement to forms with diffuse skin sclerosis and severe and often progressive internal organ involvement, and occasionally a fulminant course. 1 The affected organs and systems include the skin, lungs, heart, digestive system, kidneys, muscles, joints, and nervous system.2
The pathogenesis of systemic sclerosis is complex and incompletely understood. Immune activation, vascular damage, and excessive synthesis of extracellular matrix with deposition of increased amounts of structurally normal collagen are all known to be important in the development of this illness. 3 Different factors, including genetic, environmental, vascular, autoimmunologic factors are involved in the pathogenesis 2 . The clinical and pathologic manifestations result from three distinct processes: 1) severe fibroproliferative vascular lesions of small arteries and arterioles, 2) excessive and often progressive deposition of collagen and other extracellular matrix macromolecules in skin and various internal organs, and 3) alterations of humoral and cellular immunity. 1
Numerous studies have suggested a sequence …show more content…
The exaggerated connective tissue production is induced by cytokines and growth factors released from the tissue-infiltrating inflammatory cells. One of the growth factors that plays an important role in the fibrosis is TGF-β. The TGF-B stimulate the extracellular matrix synthesis and induces the generation of myofibroblasts. TGF-β also decreases the production of collagen-degrading metalloproteinases and stimulates the production of protease inhibitors, which prevent extracellular matrix breakdown.