Systemic sclerosis (SSc) is a disease of systemic connective tissue. Systemic sclerosis characteristics include essential vasomotor disturbances; fibrosis; subsequent skin atrophy (see picture below), subcutaneous tissue, muscles, and internal organs (e.g., dietary tract, lungs, heart, kidney, CNS); and immunological disturbances accompany such findings.
• Limited SSc (CREST syndrome)
• Generalized SSc (with diffuse skin involvement)
• SSc sine scleroderma
Sometimes systemic sclerosis worsens rapidly and becomes fatal (mainly with generalized systemic sclerosis). At other times, it affects only the skin for decades before affecting internal organs, although some damage to internal organs (such as the esophagus) is almost inevitable. The course is unpredictable.
Currently, there is no cure for scleroderma, but there are many treatments available to help particular symptoms. For instance, heartburn can be controlled by medications called proton pump inhibitors PPIs) or medicine to improve the motion of the bowel. Some treatments are directed at decreasing the activity of the immune system. Some people with mild disease may not need medication at all and occasionally people can go off treatment when their scleroderma is no longer active. Because there is so much variation from one person to another, there is great variation in the treatments prescribed.