Scleroderma is a rare and chronic condition where the immune system mistakenly targets the body’s own tissues, causing inflammation and scarring. The condition is characterized by thickening and hardening of the skin, but can also affect internal organs, depending on the type of scleroderma.
There is currently there is no cure for scleroderma, but there are treatments to manage its symptoms and therapies for conditions associated with scleroderma. Potential new therapies are under development.
Treatment for skin symptoms
Patches of hardened skin may be treated with topical medications to soften and reduce stiffness. These include creams and ointments containing calcipotriol, tacrolimus, imiquimod, or steroids.
Digital ulcers — sores on the fingers and toes — are a common symptom of scleroderma that can cause extreme pain and infection if left untreated.
There are several approved and off-label treatments for scleroderma patients with digital ulcers. In Europe, Ventavis (iloprost) and Tracleer (bosentan) are commonly used to treat and prevent digital ulcers, but these medications are not yet approved in the U.S.
Pain relief medications
Scleroderma can cause chronic pain in the muscles, joints, and tendons. In cases where common over-the-counter pain medications are not sufficient, stronger pain relievers may be recommended, such as Ultram (tramadol).
In extreme cases, narcotic analgesics such as Vicodin (acetaminophen/hydrocodone), Percocet (acetaminophen/oxycodone), and OxyContin (oxycodone hydrochloride) may be prescribed. These should not be used over a long period of time because they are very addictive.
Anti-inflammatory agents
Scleroderma is associated with chronic inflammation, which can lead to damage and pain. There are several therapies that can reduce inflammation. These can also help treat muscle and joint pain caused by the condition. Examples include:
- Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen;
- COX-2 inhibitors such as Celebrex (celecoxib);
- Corticosteroids such as prednisone and Medrol (methylprednisolone).
Anti-fibrotics
Fibrosis, or scarring, caused by scleroderma involves the over-production of collagen protein that builds up and damages the skin and other organs.
Anti-fibrotic therapies work on a part of the immune system that is involved in damage response by reducing collagen production, which is needed for scar tissue formation.
There are several anti-fibrotic therapies used in scleroderma, but there is a lack of robust clinical trial data to support their use. Historically, D-penicillamine has been used as an anti-fibrotic treatment for scleroderma with varying degrees of success, but its effectiveness has been debated.
Other examples, mainly used in pulmonary fibrosis, include Esbriet (pirfenidone) and Ofev (nintedanib).
Immunosuppressants
The symptoms of scleroderma are triggered by an abnormal immune response. Therefore, suppressing the immune system can prevent the damaging responses such as inflammation and fibrosis.
There are several immunosuppressant therapies used to treat scleroderma, although the benefit of these therapies in scleroderma has not been supported by many large-scale or placebo-controlled trials.
Examples include cyclosporine, CellCept (mycophenolate mofetil), Imuran (azathioprine), methotrexate, and Thymoglobulin (anti-thymocyte globulin).
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