The pathogenesis of systemic sclerosis

Systemic sclerosis (SSc) is an uncommon, complex, multisystemic disorder, characterized by severe fibrosis of the skin and various internal organs. As in other collagenoses, the etiology of SSc is unknown and the exact mechanisms involved in the pathogenesis are not well understood. For the clinical and pathogenetic manifestations, however, the following key processes have been identified: 1) excessive accumulation of collagen and other components of the extracellular matrix; 2) early morphological changes in small blood vessels; and 3) alterations in the cellular and humoral immune response resulting in the production of disease-specific antibodies. Currently, it remains unclear how these processes interact, to cause a chronic and progressive fibrotic disease. Continued research, has however yielded substantial insight into the molecular understanding of several basic mechanisms, suggesting novel therapeutic targets for the future.