Scleroderma - Treatment

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What is the treatment for scleroderma?

Treatment of scleroderma is directed toward the individual features affecting different areas of the body.

Aggressive treatments of elevations in blood pressure have been extremely important in preventing kidney failure. Blood pressure medications, particularly the angiotensin converting enzyme (ACE) inhibitor class of drugs, such as lisinopril, are frequently used.

Some research indicates that colchicine can be helpful in decreasing the inflammation and tenderness that periodically accompanies the calcinosis nodules in the skin. Skin itching can be relieved with lotions (emollients) such as Eucerin, Lubriderm, and Bag Balm.

Mild Raynaud's phenomenon may require only hand warming and protection. Low-dose aspirin is often added to prevent tiny blood clots in the fingers, especially in patients with a history of fingertip ulcerations. Moderate Raynaud's phenomenon can be helped by medications that open up the arteries, such as nifedipine (Procardia, Adalat), nicardipine (Cardene), and diltiazem (Cardizem), or with topical nitroglycerin applied to the most affected digit (most effective on the sides of the digit where the arteries are). Gently applied finger splinting can protect tender tissues. (It is important to not constrict the tiny arteries on the sides of the fingers when protecting them with splints, braces, or band aid materials.) A class of medications that is typically used for depression, called serotonin reuptake inhibitors, such as fluoxetine (Prozac), can sometimes improve the circulation of the affected digit. Drugs that constrict blood vessels, such as pseudoephedrine (Sudafed decongestant) should be avoided. Severe Raynaud's phenomenon can require surgical procedures, such as those to interrupt the nerves of the finger that stimulate constriction of the blood vessels (digital sympathectomy). Ulcerations of the fingers can require topical or oral antibiotics.

Esophagus irritation and heartburn can be relieved with omeprazole (Prilosec), esomeprazole (Nexium), or lansoprazole (Prevacid). Antacids can also be helpful. Elevating the head of the bed can reduce the back flow of acid into the esophagus that causes inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps. Of note, there is an increased risk of developing lung cancer in persons with scleroderma. This risk is even greater in those who smoke.

Constipation, cramping, and diarrhea are sometimes caused by bacteria that can be treated with tetracycline, erythromycin, ciprofloxin, or metranidazole. Increased fluid intake and fiber intake are good general measures. A variety of medications that promote movement of the bowels are available.

Irritated, itchy dry skin can be helped by emollients such as Lubriderm, Eucerin, Bag Balm, histamine-2 blockers, or trazodone (Desyrel).

Telangiectasias, such as those on the face, can be treated with local laser therapy. Sun exposure should be minimized as it can worsen telangiectasias.

Approximately 10% of patients with the CREST variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension). Abnormally elevated blood pressure of the arteries supplying the lungs is often treated with calcium antagonist medications, such as nifedipine (Procardia), and blood-thinning drugs (anticoagulation). More severe pulmonary hypertension can be helped by continuous intravenous infusion or inhalation of prostacyclin (Iloprost). Taken by mouth, bosentan (Tracleer) is used to treat pulmonary hypertension. In addition, sildenafil (Revatio) and tadalafil (Cialis) have been FDA approved to treat pulmonary hypertension.

Additionally, medications are used to suppress the overly active immune system that seems to be spontaneously causing the disease in organs. Medications used for this purpose include penicillamine, azathioprine (Imuran, Azasan), and methotrexate (Rheumatrex, Trexall). Serious inflammation of the lungs (alveolitis) can require immune suppression with cyclophosphamide (Cytoxan) along with prednisone (Deltasone, Liquid Pred). The optimal treatment of scleroderma lung disease is an area of active research. Stem cell transplantation is being explored as a possible option.

No medication has been found to be universally effective for all patients with scleroderma. In an individual patient, the illness may be mild and not require treatments. In some, the disease is ravaging, relentless, and can lead to death. Because blood vessel damage is a primary part of the pathology of scleroderma, patients with this disease must not smoke.

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See what others are saying

Comment from: Bug43, 65-74 Female (Patient) Published: April 15

I've had diffuse scleroderma since 2009 and have been able to successfully manage my disease and symptoms with minocycline. I also take vitamin D3, which helped my muscle spasms. I started with 100 mg twice a day. Now I take 100 mg twice a day, every other day.

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