Scleroderma is a disease of the skin, joints and internal organs. Its cause is not known. Scleroderma means hardening (fibrosis) of the skin, and due to malfunction of the vascular and immune systems, resulting in the overproduction of collagen (connective tissue in the body). Scleroderma can be localized to the skin or may become systemic with excess collagen building up in various organs such as the esophagus, kidneys, lungs, gastrointestinal tract, heart skin and peripheral nervous system.
Fortunately, scleroderma is relatively rare, affecting approximately 300,000 persons in the United States. The disorder most commonly occurs in women between the ages of 20 and 40—however, men and children can be affected as well. The disease is not contagious and is not thought to be inherited. Currently, there is no know cure for scleroderma. Early diagnosis is important to enable initiation of treatment to prevent long-term complications. The primary goals of treatment are to relieve and control symptoms, improve quality of life, and prevent long-term complications.
Treatment of Scleroderma
- Therapy: There’s no cure for every patient with scleroderma, though there’s treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.
- Skin changes: If you have localized scleroderma, your doctor may recommend a topical treatment, such as a moisturizer or corticosteroid medication that you apply to your skin. Corticosteroid medications impede your body’s ability to make substances that can cause inflammation.
If your condition involves a large area of skin, your doctor may recommend additional treatments. Doctors sometimes prescribe minocycline (Minocin, Dynacin) to control the skin-related (cutaneous) symptoms of scleroderma, although no studies have addressed its long-term effectiveness. In preliminary studies, light therapy (phototherapy) has also proved effective in treating the lesions that are associated with scleroderma, but more research is needed.
Cosmetic treatments are another consideration. Some people with scleroderma are discouraged or embarrassed by lesions and marks on the skin, including tiny dilated blood vessels that often appear on the face (telangiectasia). Specialized brands of foundation makeup and pulsed dye laser surgery can help camouflage or eliminate these lesions. Consult a dermatologist about treatments for skin changes.
- Joint stiffness, pain and inflammation: Your doctor may prescribe anti-inflammatory medications such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs) like naproxen or low-dose corticosteroids to relieve joint pain and stiffness. Topical treatment for the skin changes of scleroderma don’t alter the disease course, but may improve pain and ulceration. There is limited benefit from steroids such as prednisone.Episodes of Raynaud’s phenomenon sometimes respond to nifedipine or other calcium channel blockers; severe digital ulceration may respond to prostacyclin analogue iloprost and the dual endothelin-receptor antagonist bosentan may be beneficial for the condition. The skin tightness may be treated systemically with methotrexate and cyclosporin.Often, along with NSAIDs, doctors prescribe certain medications called disease-modifying antirheumatic drugs (DMARDs). These medications seem to do their job by having an effect on immune systems that have gone out of control, but doctors don’t understand exactly how DMARDs work. Common DMARDs include:Hydroxychloroquine (Plaquenil): This drug has relatively few side effects and is also effective for the arthritis that can be associated with scleroderma. Apart from hydroxychloroquine’s apparent ability to affect the way immune cells work, scientists don’t completely understand how it helps tame the disease process.Penicillamine (Cuprimine, Depen): Similar to other DMARDs, penicillamine can reduce inflammation. Its full effect may require many months to develop, but its beneficial effects may be longer lasting. However, because of a relatively high incidence of adverse reactions to this drug and studies casting doubt on its effectiveness, its use has declined in recent years.
Methotrexate (Rheumatrex, Trexall): This drug does its job by affecting cells that are responsible for some of the pain, inflammation and joint swelling that accompany scleroderma. Trials have shown conflicting results regarding the effectiveness of methotrexate in treating the disease.
Immunosuppresents are another class of medications that can help manage out-of-control immune systems. Cyclophosphamide (Cytoxan) is one example. This extremely potent medication works by damaging cells’ genetic information. In particular, it kills white blood cells called lymphocytes that are part of the autoimmune disease.
- Circulation problems: Your doctor may also prescribe medications to dilate blood vessels and promote circulation. These medications can prevent high blood pressure and kidney problems and help treat Raynaud’s phenomenon. Medications that help with blood circulation include Calcium channel blockers, Alpha blockers, Angiotensin-converting enzyme (ACE) inhibitors, Angiotensin II receptor blockers and low-dose enteric-coated aspirin. Creams containing nitroglycerin may also help promote circulation.
- Kidney disease: Scleroderma renal crisis—the occurrence of acute renal failure and malignant hypertension or very high blood pressure with evidence of organ damage in people with scleroderma—is effectively treated with drugs from the class of the ACE inhibitors. The benefit of ACE inhibitors extends even to those who have to commence dialysis to treat their kidney disease, and may give sufficient benefit to allow the discontinuation of renal replacement therapy.
- Lung disease and pulmonary hypertension: If you have scleroderma that affects your lungs, you may need additional medications. Cyclophosphamide (Cytoxan) is sometimes used to treat pulmonary fibrosis. A 2006 study of people with scleroderma-related lung disease found cyclophosphamide modestly improves lung function and quality of life. The long-term effects of cyclophosphamide treatment in people with scleroderma are unknown.Bosentan (Tracleer) is an oral medication that has been approved for pulmonary hypertension in people with scleroderma. Pulmonary hypertension may be treated with epoprostenol, bosentan and possibly aerolized iloprost.
- Digestive difficulties: If scleroderma has affected your esophagus and you’re experiencing heartburn, your doctor may suggest prescription medications that decrease stomach acid production. These medications include H-2 receptor blockers and proton pump inhibitors. Your doctor may also suggest antibiotics, special diets and medications that improve your gut’s ability to contract.
- Experimental treatments: Given the difficulty in treating scleroderma, treatments with a smaller evidence base are often tried to control the disease. These include antithymocyte globulin and mycophenolate mofetil; some reports have shown improvements in the skin symptoms as well as delaying the progress of systemic disease, but neither of them have been subjected to large clinical trials.
While still experimental given its high rate of complications, hematopoietic stem cell transplantation is being studied in patients with severe systemic sclerosis; improvement in life expectancy and severity of skin changes has been duly noted.
Causes of Scleroderma
Again, there’s no clear, obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited, and genetic concordance is small; still, there’s often a familial predisposition for autoimmune disease. Polymorphisms in COL1A2 and TGF-β1 may influence severity and development of the disease. There’s limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.
One of the suspected mechanisms behind the autoimmune phenomenon is the existence of microchimerism—i.e., fetal cells circulating in maternal blood—triggering an immune reaction to what is perceived as “foreign” material. A distinct form of scleroderma and systemic sclerosis may develop in patients with chronic renal failure. Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis has been linked to the exposure to gadolinium-containing radiocontrast. Bleomycin, (a chemotherapeutic agent) and possibly taxane chemotherapy, may cause scleroderma, while occupational exposure to solvents has been linked with an increased risk of systemic sclerosis
Anyhow, now that you know most everything you need to know about treating and handling scleroderma, you should be all right. Just pick the best option for you or, better yet, consult your doctor for a more in-depth look into the disease—your body will thank you for it.
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