The widespread clinical presentations and complicated organ involvement make management of systemic sclerosis the most challenging among autoimmune diseases. Newer therapeutic drugs aim to target the disease process by halting its progression and reversing the damage. Most of these are still under clinical evaluation. Intolerable side effects and variable efficacies limit the widespread use of newer agents. Systemic sclerosis is a chronic autoimmune disorder of unknown etiology that affects the skin and internal organs.
Progressive thickening and fibrosis of the skin occurs. Multiple internal organs, most commonly the lung, are involved. Pathogenesis of systemic sclerosis involves the excessive production of collagen by activated fibroblasts leading to fibrosis and microvasculopathy. Antibodies targeting RNA polymerases, topoisomerases and kinetochore have been described in systemic…
