Scleroderma

The chronic disease scleroderma, also called systemic sclerosis, affects about 300,000 Americans. It’s a chronic connective tissue disease that can be difficult to properly diagnose. Scleroderma often—but not always—involves hardening and tightening of the skin and other connective tissue, which is where the condition gets its name. Scleroderma can also affect internal organs. Other symptoms include gastrointestinal reflux when the intestinal muscles don’t function properly in the digestive process, and pain and numbness in the extremities in cold weather or as a reaction to strong emotion due to constriction of blood vessels.

Scleroderma can happen at any age, but mostly between 30 and 50, and is more common in women. For reasons that aren’t clear, Choctaws living in Oklahoma are particularly prone to the condition, but those in Mississippi are not. This geographic distinction has not been seen in other ethnic groups. African-Americans are more likely to develop scleroderma than people of European descent. Moreover, people who are exposed to silica dust or certain industrial solvents have an elevated risk of the production of excess collagen in tissue that causes the illness. In general, however, there seems to be an autoimmune component to the condition.

There is no cure for scleroderma, but immunosuppressants can be used to manage the condition, and blood pressure medications can help alleviate the pain from vasoconstriction. Physical and occupational therapy are used to help patients with pain and mobility. The disease can cause stiffness, and therapies can help counteract this. Scleroderma patients can avoid the worst complications by staying active, keeping warm, and not smoking.

Unfortunately, in addition to being underdiagnosed, there is some indication that mortality rates from scleroderma are significantly underestimated. Survival data had reportedly been collected without regard to how long patients had had the disease, meaning that the data set was skewed by including long-term patients and people who had been diagnosed less than five years in the same grouping. That means that long-term survival rates were overestimated because people who did not survive long-term were largely excluded from the patients being studied.

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