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Scleroderma - systemic sclerosis

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Scleroderma has no known cure — there is no treatment to stop the overproduction of collagen. Your doctor may recommend a number of medications to make it easier for you to live with scleroderma by treating its symptoms. Your doctor may also suggest treatments to deal with complications of scleroderma that may affect various organs.

Doctors often treat localized scleroderma with therapies such as moisturizers or corticosteroid medications that you apply to your skin. Corticosteroid medications impede your body's ability to make substances that can cause inflammation.

If the condition involves a lot of body area or an arm or a leg, your doctor may prescribe systemic drugs to try to halt the progression of the disease. Treatment of systemic scleroderma may be with drugs that improve blood flow, promote esophagus and bowel function, preserve kidney function and control high blood pressure.

Raynaud's phenomenon
To dilate blood vessels and promote circulation, your doctor may prescribe:

  • Calcium channel blockers. These medications help relax blood vessel muscles, and some slow your heart rate. They include amlodipine (Norvasc), diltiazem (Dilacor, Tiazac), nifedipine (Adalat, Procardia) and verapamil (Calan, Verelan).

  • Alpha blockers. These medications prevent muscle contractions in smaller arteries and reduce the effects of naturally occurring body chemicals that narrow blood vessels. Alpha blockers include doxazosin (Cardura).

  • Angiotensin-converting enzyme (ACE) inhibitors. ACE inhibitors help relax your blood vessels by blocking the formation of a natural chemical inside your body that narrows blood vessels. These medications include captopril (Capoten), benazepril (Lotensin), lisinopril (Prinivil, Zestril), ramipril (Altace) and enalapril (Vasotec).

  • Angiotensin II receptor blockers. These medications act in a manner similar to that of ACE inhibitors, but they block the action of the chemical instead of the formation of the chemical itself. They include losartan (Cozaar).

  • Low-dose enteric-coated aspirin. Aspirin reduces your blood's clotting action and helps keep your blood vessels open. The coating on the aspirin prevents the release and absorption of the aspirin until the pill reaches your intestines.

Creams containing nitroglycerin also may help promote circulation.

Joint stiffness and pain
Your doctor may prescribe anti-inflammatory medications such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs) or low-dose corticosteroids.

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. No one knows for sure how DMARDs work.

Common DMARDs include:

  • Hydroxychloroquine (Plaquenil). Originally developed as a treatment for malaria, this drug has relatively few side effects. 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). Similar to other DMARDs, penicillamine can reduce inflammation, but you must be patient. Its full effect may require many months to develop. However, its beneficial effects may be longer lasting. 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.

Another class of medications, immunosuppressants, is able to tame out-of-control immune systems. Some of these drugs are cytotoxic, meaning they attack and eliminate cells associated with the disease. Some immunosuppressants your doctor may prescribe include:

  • Methotrexate (Rheumatrex). 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 scleroderma.

  • Cyclophosphamide (Cytoxan). This extremely potent medication works by damaging cells' genetic information. In particular, it kills lymphocytes that are part of autoimmune disease. Cyclophosphamide may be used to treat lung inflammation.

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. Prescription-strength H-2 receptor blockers include nizatidine (Axid), famotidine (Pepcid), cimetidine (Tagamet) and ranitidine (Zantac). Proton pump inhibitors include lansoprazole (Prevacid), omeprazole (Prilosec), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Your doctor may also suggest antibiotics, special diets and medications that improve your gut's ability to contract.

Kidney complications and high blood pressure
The best way to prevent kidney complications is to control high blood pressure. To accomplish this, your doctor may prescribe ACE inhibitors or angiotensin II receptor blockers.

Pulmonary hypertension
Your doctor may prescribe the oral medication bosentan (Tracleer) if you have moderately severe pulmonary hypertension. This medication may improve your ability to exercise and may slow the progression of pulmonary hypertension. However, pregnant women shouldn't take this drug. Side effects may include liver damage.

Skin-related (cutaneous) symptoms
Doctors sometimes prescribe minocycline (Minocin, Dynacin) to control the cutaneous symptoms of scleroderma, although no studies have addressed its long-term effectivenesss.

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