An Overview of Systemic Sclerosis

Systemic sclerosis (SSc, scleroderma) is an uncommon connective tissue disorder with complex and poorly understood pathogenesis. It is characterized by prominent vascular alterations with endothelial cell damage and proliferation of subendothelial connective tissue, skin fibrosis and involvement of the lungs, gastrointestinal tract, heart, kidneys and central nervous system. Immune disturbances and production of general and specific autoantibodies accompany these disorders. Systemic sclerosis affects women more than men and age of onset varies between 20 and 60 years. On the basis of skin involvement, SSc is divided into two major categories limited cutaneous SSc, which is usually associated with mild to moderate, delayed organ fibrosis and diffuse cutaneous SSc, which is characterized by progressive skin indurations, starting in the fingers and ascending to proximal extremities. Patients with this disorder are at risk for early pulmonary fibrosis and acute renal involvement. Face might be involved in both diffuse and limited cutaneous SSc. The cause of SSc is not fully clarified but data analysis show that infectious agents, environmental toxins, drugs, epigenetic modifications and microchimerism, might be potential causal agents in a genetically susceptible individual. This article presents an overview of the SSc disorder, the many facets of a very complex, disabling disease, affecting patients quality of life and life expectancy.