How should I be treated for scleroderma?

The Scleroderma Research Foundation cannot provide specific medical advice, only a qualified doctor is able to evaluate a patient properly and provide an accurate diagnosis.

If a patient has systemic scleroderma, the most common symptoms are:

Raynaud’s Phenomenon: White/blue/red fingers and toes very sensitive to cold
Treatments include ACE inhibitors and calcium channel blockers. ACE inhibitors block the formation of an enzyme in the body that narrows blood vessels. This causes the blood vessels to relax and lowers blood pressure, increasing the supply of blood and oxygen to the heart. Calcium channel blockers affect the movement of calcium into the cells of the heart and blood vessels, increasing the supply of blood and oxygen to the heart while reducing the heart’s workload. The drugs can relax blood vessel muscles and may slow the heart rate.

Some side effects of ACE inhibitors include swelling of face, mouth, hands or feet; dizziness, lightheadedness or fainting, skin rash, fever or joint pain. Some side effects of calcium channel blockers include aggravated acid reflux, swelling of ankles, feet or lower legs.

Swollen or puffy fingers (Sclerodactyly)
Treatments include non-steroidal inflammatory drugs (NSAIDs), analgesics and/or steroids. These drugs relieve joint, muscle and inflammatory pain by blocking substances in the body that cause inflammation and pain. The NSAIDs are "COX-2 inhibitors." Analgesics include aspirin. Because of the potential side effects of steroids, many doctors are reluctant to prescribe steroids.

Some side effects of NSAIDs include abdominal or stomach cramps, pain or discomfort (mild to moderate) and diarrhea (if taking mefenamic acid, stop medication and check with a doctor immediately).

Rare side effects of analgesics include bloody or black, tarry stools; bloody or cloudy urine; fever with or without chills. Check with a doctor as soon as possible if you or someone you know is experiencing any of these symptoms.

Corticosteroids are very strong medicines. In addition to their potential helpful effects, they have side effects that can be very serious. Glucocorticosteroids, also sometimes used to treat pulmonary fibrosis, have been found to increase risk of scleroderma renal crisis and should be used with caution and only in select cases under close observation such as frequent blood pressure checks. Be sure to discuss the risks and benefits of this medicine with a qualified physician. Corticosteroids may lower resistance to infections, and any infection may be harder to treat. Always check with a doctor as soon as possible if you or someone you know experience any signs of a possible infection, such as sore throat, fever, sneezing or coughing.

Some side effects from short term use of steroids include increased appetite, indigestion, nervousness or restlessness. Less common or rare are darkening or lightening of skin color, dizziness or lightheadedness, flushing of face or cheeks, increased joint pain (after injection into a joint), increased sweating and a sensation of spinning.

Some side effects from long term use: The most common side effects from long-term use of corticosteroids are central obesity, Cushing’s Syndrome, osteoporosis, moon facies (fay-SEEZ), buffalo hump and striae.

Heartburn, difficulty swallowing (Esophageal dysfunction)
Treatments include proton-pump inhibitors, H-2 blockers or antacids. Proton pump inhibitors decrease stomach acid production, minimizing gastrointestinal reflux disease, and help prevent esophageal ulcers. H-2 blockers work by decreasing the amount of acid produced by the stomach. Antacids work by neutralizing excess stomach acid to relieve heartburn, sour stomach or acid indigestion.

Some side effects of proton pump inhibitors, though uncommon, include headache, diarrhea and/or constipation.

Some side effects of H-2 blockers, though uncommon, include constipation, diarrhea, difficult urination, dizziness, drowsiness, dryness of mouth or skin and/or headache.

Some side effects of antacids include chalky taste; less common side effects are mild constipation, diarrhea and increased thirst.

High blood pressure in the lungs; shortness of breath, chest pain, dizziness (Pulmonary Arterial Hypertension or PAH)
If you or someone you know experience any of the above symptoms of pulmonary hypertension, see a doctor immediately.

Treatments include prostaglandins, prostacyclins, endothelin receptor antagonists. Prostaglandins and prostacylins work by relaxing blood vessels and increasing blood to the lungs. Endothelin receptor antagonists work by blocking the vessel-narrowing hormone, endothelin, found in the blood and lungs. They increase the supply of blood to the lungs and reduce the workload of the heart.

Some side effects of prostaglandins include diarrhea, fast heartbeat, headache, lightheadedness or fainting, nausea, redness of face or neck (flushing) and/or vomiting.

Side effects of prostacyclins include headache, cough, flushing, jaw pain and influenza-like symptoms.

Some side effects of endothelin receptor antagonists: Blurred vision, confusion, dizziness, dark urine, faintness or lightheadedness, fever and/or chills. Also, it is important to carefully monitor liver function when using this class of drugs.