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The Initial Effects of Diffuse Scleroderma

By Philip Clements, M.D., M.P.H., UCLA School of Medicine, Dept. of Rheumatology (originally published in Scleroderma Voice, 2002 #2)

Philip Clements, M.D., M.P.H.

Philip Clements , M.D., M.P.H.

Question: Is it true that the effects of diffuse scleroderma start at the top of the body and travel down?

Answer: Scleroderma usually begins as one of three complaints:

(1) puffy hands;

(2) Raynaud's phenomenon; or

(3) joint pain or arthritis.

Diffuse scleroderma tends to affect the hands first, then moves up the arm (and feet and legs in some people) to involve the upper arms, the thighs, the belly, and the chest. The face is usually affected early in the illness.

To determine the risk of possible internal involvements in diffuse scleroderma, we need to know how long the person has had scleroderma since it first began (usually the time when one of those initial three manifestations I mentioned above occurred).

If it has been more than 5 years since the onset of scleroderma, and there is not yet significant heart, lung, or kidney involvement, it is unlikely that new heart, lung or kidney involvement will begin after this time.

If someone is still within the first one or two years of scleroderma onset, there is potential for heart, lung, and kidney involvement. This needs to be screened for by patient and physician.

Gut involvement tends to have its own timetable, and does not really tell us much about the rest of the disease course or the risk of other internal involvements.

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