Identification of a number of antibodies present in SS patients has not made a major impact on an understanding of the pathogenesis of the disease, but it has focused investigation on the contribution of the immune response to the vascular injury and resultant fibrosis. Thus, it now seems clear that T cell hyperactivity occurs in SS patients. The stimulus for T cell hyperactivity is not known. Autoimmunity to laminin is seen in SS patients. Monocytes also are probably major players in the pathogenesis of SS, especially as they share the same cellular lineage as endothelial cells, and are major participants in antigen presentation and in cytokine activation and release.
Monocytes do secrete active TGFb, a major fibrogenic cytokine.
Patients older than 40-50 years have significantly reduced survival rates. The key to success of stem cell transplantation for autoimmune disease will be patient selection. Choosing appropriate patients with systemic sclerosis for treatment by autologous stem cell transplantation.