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The systemic forms of scleroderma cause fibrotic (scar) tissue to be produced in the skin and internal body organs. Problems occur because the scar tissue builds up in the skin and the internal organs and interferes with the normal function of these organs. Since systemic scleroderma is a multiorgan disease, treatment must be targeted to each of the potential manifestations of the disorder such as Raynaud's phenomenon, gastrointestinal complications, pulmonary complications, and cardiac complications. Therapy for systemic scleroderma includes medication for the condition as well as treatment targeted at individual aspects that are affected.

Although no single therapy has been proven to reverse the fibrosis and vascular damage causes by systemic scleroderma, several drugs are available to slow the progression of the disease and improve the patients quality of life. As is the case for localized scleroderma, many of the drugs used by physicians for the management of patients with systemic scleroderma have not been proven to be effective in controlled clinical trials and, therefore, patients should be monitored carefully for adverse side-effects as well as to determine if the patient's clinical condition is improving.

In general, the following categories of drugs may be used for the treatment of systemic scleroderma:

• Immunosuppressive agents
• Antifibrotic agents
• Anti-inflammatory agents
• Angiotensin-converting enzyme inhibitors

Immunosuppressive Agents

A variety of drugs that suppress the immune system have been used for the treatment of systemic scleroderma. Controlled clinical trials have yielded mixed results on many of these drugs and further investigation continues.

Immunosuppressive drugs are used for the treatment of lung complications of systemic scleroderma including pulmonary fibrosis and aleveolitis. They may also be used to treat skin fibrosis. Examples of immunosuppressive drugs that may be used include:

• Cyclophosphamide (Cytoxan)
• Purine antimetabolites such as 6-thioguanine and azathioprine (Imuran)
• Cyclosporine (Neoral; Sandimmune)
• Mycophenolate mofetil (Cellcept)
• Chlorambucil (Leukeran)
• Methotrexate (Trexall; Rheumatrex)

Antifibrotic Agents

Antifibrotic agents are medications that are used to attempt to reduce the formation of collagen. Here too, results are mixed in terms of effectiveness, dose recommendations and effectiveness. Examples of antifibrotic agents that may be used for the treatment of systemic scleroderma include:

• D-penicillamine (Cuprimine; Depen) - This drug is effective in some patients at certain doses in softening the skin, slowing the progression of organ involvement, and increasing survival.
• Colchicine - one study involving a small number of patients indicated effectiveness in improving skin elasticity, mouth opening, dysphagia and finger flexibility.
• Interferon gamma has been shown in limited clinical trials to result in a modest improvement of skin fibrosis and slowing the progression of organ involvement in patients with systemic scleroderma.
• Anti-transforming growth factor beta - This drug is currently being investigated as a possible new treatment for systemic scleroderma.

Anti-Inflammatory Agents

Several anti-inflammatory drugs may be used to reduce the inflammation and joint and tendon swelling associated with systemic scleroderma including:

• Aspirin - Examples include Anacin, Bayer, Bufferin, among others.
• Non steroidal anti-inflammatory drugs (NSAIDs) - Examples include ibuprofen (Motrin, Advil), indomethacin (Indocin), naproxen (Naprosyn, Aleve), and ketoprofen (Orudis).