Scleroderma

Literally “hard skin,” scleroderma is also called “systemic sclerosis.”  Like all auto-immune diseases, the cause is unknown.  It may begin with puffy or shiny skin on the hands or face, but eventually skin gets stiff.  This causes pain with movement, weakness, and general fatigue.  Joints don’t get inflamed, but get tight and can’t move.  A common complication is Raynaud phenomenon, when exposure to cold makes the fingers hurt and turn red, white, and blue.

Diagnosis begins by suspecting the condition because of skin changes, mainly in the hands and face.  Sometimes there are small ulcers around the fingertips, lips may be tight, gums may recede, and small purple star-like spots may come out on the skin or gums (“telangiectasias”).

The first blood test to order is the Antinuclear Antibody (ANA).ย  If that’s negative, scleroderma is very unlikely.ย  If it’s positive, we refer to a rheumatologist, who may get other antibody tests, with names like Antitopoisomerase I (anti-Scl-70), Anticentromere antibody (ACA), Anti-RNA polymerase III, and maybe more.

The problem with Scleroderma is that internal organs may gradually become affected.ย  These include swallowing difficulties, sexual dysfunction, gradual loss of lung function, gradual loss of kidney function, and sometimes more rapid loss of heart function.ย  Tests get done regularly on the lungs, kidneys, and heart.ย  Treatments exist, but they’re not as helpful as they are for the other various types of arthritis and rheumatologic conditions.

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