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Doctors' Answers to "Frequently Asked Questions" - Scleroderma
Answer: Sounds like scleroderma which can be occasionally diagnosed by biopsy of the tissue. Hard to know if any treatment is effective for this disease. Steroids would clearly be used in the US, methotrexate and d-penicillamine would usually not unless there were renal or pulmonary involvement which you do not mention. She should have pulmonary function testing with diffusing capacity(CO) performed as a baseline and done every 3-4 months. These patients also sometimes belong to a group called CREST. This is a combination of tissue calcinosis, reynauds phenomenon, esophageal motility problems, scleroderma and telangiectastas. Treatment is usually frustrating in this type of disease. But, the drugs she is on are clearly in the right direction, I would ensure there are some markers for the severity of the disease to be able to decide in a few months if the treatment is having any effect.
Answer: Not usually, but each case is individual and the speed of progression varies markedly.
Answer: I am not sure from your description as to the location of the pain. That is, is this a nerve compression problem, scarring from surgery, or changes due to scleroderma. A pain specialist or neurologist might be able to help you figure this one out.
Answer: Scleroderma is also referred to as sytemic sclerosis. This is a disease with unknown
cause which is grouped in the category of connective tissue diseases. It is thought to be a
disorder of the immune system "attacking" ones own connective tissues. It usually affects
multiple organs in the body with thickening or fibrosis. Women are affected about three
times more often than men and the disease is usually seen starting at age thirty-and then more
frequently with age. The skin becomes thickened and leathery the Gl tract thickens and
motility is a problem-especially the esophagus, thickening of the renal system can cause renal
failuse, thickening of the lung tissue-respiratory difficulties, and thickening of the heart
muscle-heart failure. The course of this disease varies a great deal. Some patients with only
minor non-progressive problems, others with rapidly progressive multi-organ failure. Drugs
don't seem especially effective although d-penicillamine has been used and steroids are
sometimes used for treatment of a specific organ. Diagnosis of this disease is usually by the
characteristic clinical picture, although biopsy is sometimes helpful.