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Although there isn’t a cure for scleroderma there are effective treatment options that help alleviate symptoms and slow down disease progression. Current prescription and over-the-counter medication are designed to treat scleroderma by targeting: 1) inflammation, 2) autoimmunity, 3) vascular disease, and 4) tissue fibrosis. As a medical doctor or health care professional caring for persons with scleroderma, it is important to ensure patients understand the nature of the medications they may be prescribed and how they work to help control disease symptoms from progressing. Patient knowledge is powerful, as it reduces fear and increases compliance, resulting in a higher percentage of successful treatment.
The following list of medication, commonly prescribed by doctors to treat patients with scleroderma, has been compiled, in thanks, by the Scleroderma Foundation. The list is provided for informational purposes only, and is not to be taken as an endorsement of any drug by the Scleroderma Society of Ontario or its Medical Advisory Board.
Many of these drugs are prescribed by doctors, but have not been proven useful and should be used only with caution and supervision. It is important to consider, that not every medication is appropriate for every patient. As a medical doctor familiar with your patient's medical history, health status and disease progression, you will be able to determine the most appropriate treatment options.
Scleroderma Medications Guide
Calcium Channel Blockers nifedipine (Procardia®, Adalat®) |
Action Relax blood vessels |
Most Common Side Effects Low blood pressure, constipation, nausea, may worsen swallowing difficulty, light-headedness, headache, leg edema, palpitations, irregular heartbeats |
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Angiotensin II Receptor Antagonists losartan (Cozaar®) |
Action Block constriction of blood vessels |
Most Common Side Effects Diarrhea, dizziness, cough, muscle pain |
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Others prazosin (Minipress®) pentoxifylline (Trental®) |
Action Relax blood vessels Unclear |
Most Common Side Effects Low blood pressure Nausea, dizziness |
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Gastroesophageal Reflux Disease (GERD) |
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Antacids Gaviscon, Maalox, Mylanta, Rolaids, Tums |
Action Neutralize stomach acidity |
Most Common Side Effects Diarrhea, constipation |
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H-2 Blockers cimetidine (Tagamet®) |
Action Inhibit stomach acid secretion |
Most Common Side Effects Mental confusion (cimetidine only), diarrhea, headache, dizziness |
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Proton Pump Inhibitors omeprazole (Prilosec®) |
Action Inhibit stomach acid secretion |
Most Common Side Effects Diarrhea, headache, dizziness |
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Others sucralfate (Carafate®) |
Action Coat esophagus & stomach; forms protective barrier |
Most Common Side Effects Constipation |
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Gastrointestinal Symptoms Improve Swallowing |
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GI Stimulants bethanecol (Urecholine®) |
Action Stimulate intestinal muscle contractions, may improve heartburn |
Most Common Side Effects Abdominal cramping, diarrhea, dizziness, headache, drowsiness |
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Gastrointestinal Symptoms Relieve Constipation |
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Bulking Agents calcium polycarbophil (FiberCon®, Fiberall®) |
Action Soften stool |
Most Common Side Effects Abdominal bloating, flatulence |
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Softening Agents docusate calcium (Surfak®, Sulfolax®) |
Action Soften stool |
Most Common Side Effects Mild abdominal cramping |
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Others lactulose (Cephulac®, Cholac®) polyethylene glycol (MiraLax®) |
Action Make bowels move |
Most Common Side Effects Abdominal cramping, flatulence Nausea, abdominal bloating, cramping, flatulence |
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Gastrointestinal Symptoms Small Intestine Dysfunction: Bacterial Overgrowth/Diarrhea Broad Spectrum Antibiotic use is the mainstay of treatment for this complication. There are many potential approaches to this therapy. Example: Antibiotics are given in 2–3 week courses followed by a 1–2 week drug holiday. Generally a few cycles of this treatment can allow for quiet periods of a few months to a few years. However, some persons may require almost continuous antibiotics. Alternating antibiotics and increasing the antibiotic-free period will decrease the development of resistant strains of bacteria. Note: Prolonged use of Broad Spectrum Antibiotics may be complicated by superinfection. |
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Broad Spectrum Antibiotics Examples include: |
Action Decrease bacterial overgrowth |
Most Common Side Effects GI upset, diarrhea, nausea, and vomiting, prolonged use may be complicated by superinfection |
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Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) flurbiprofen (Ansaid®) |
Action Suppress inflammation |
Most Common Side Effects GI upset, stomach ulcers, impaired kidney function, liver inflammation, confusion
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COX-2 Inhibitors celecoxib (Celebrex®) |
Action Suppress inflammation |
Most Common Side Effects Headache, edema, rash |
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Analgesics acetaminophen (Tylenol®) tramadol (Ultram®) |
Action Relieve pain |
Most Common Side Effects Risk of liver & kidney damage Esophagus irritation & sedation |
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Narcotics acetaminophen/propoxyphene (Darvocet-N®) |
Action Relieve severe pain |
Most Common Side Effects Addiction possible, constipation, dizziness, drowsiness, nausea, vomiting |
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Immunosuppressants (none proven in controlled trials; cyclophosphamide (Cytoxan®) azathioprine (Imuran®) |
Action
Suppress immune response |
Most Common Side Effects
Bone marrow damage, hair loss, nausea, diarrhea, bladder inflammation, increased cancer risk, increased infection |
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Pulmonary Arterial Hypertension Patients diagnosed with pulmonary arterial hypertension should be referred to a pulmonary hypertension center for experienced evaluation and management of this serious scleroderma complication. |
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Endothelin Receptor Antagonists ambrisentan (Letairis®)
bosentan (Tracleer®) |
Action Act on blood vessels
Act on blood vessels |
Most Common Side Effects Edema, and birth defects
Potential liver injury, headache, flushing, edema, anemia, male infertility, birth defects |
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Prostaglandin Derivatives epoprostenol (Flolan®)
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Action Act on blood vessels
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Most Common Side Effects Headache, flushing, jaw pain, diarrhea, foot/bone pain, potential serious infection associated with central line catheter in the chest wall Flushing, increased cough, low blood pressure, headaches, nausea, spasm of jaw muscles, fainting Headache, flushing, pain at infusion site |
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Calcium Channel Blockers Can be helpful to a small proportion of patients; see Raynaud’s section |
Action |
Most Common Side Effects |
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Phosphodiesterase Type 5 (PDE5) Inhibitor sildenafil citrate (Revatio®)
tadalafil (Adcirca®) |
Action
Act on blood vessels |
Most Common Side Effects
headache, stomach upset, |
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ACE Inhibitors captopril (Capoten®) |
Action Block constriction of blood vessels |
Most Common Side Effects Skin rash, dry mouth, taste disturbances, fluid retention, drowsiness, light-headedness, diarrhea, cough |
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Warning About Corticosteroids and Renal Crisis Corticosteroids, such as prednisone and medrol, have been used to treat several complications of systemic scleroderma, such as pulmonary fibrosis, pruritis (itchy skin), and joint pain. Recent findings indicate that corticosteroids can be implicated in precipitating renal crisis. Therefore, the medical community has concluded corticosteroids should be used cautiously, in a carefully screened patient population. For example: to manage patients who also have myositis (muscle inflammation). Anyone taking corticosteroids should monitor blood pressure frequently, and report changes to the healthcare provider immediately. |
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Immunosuppressants (none proven in controlled trials; cyclophosphamide (Cytoxan®) cyclosporine (Neoral®), Sandimmune®) D-penicillamine (Cuprimine®, Depen®) methotrexate (Rheumatrex®, Trexall®) mycophenolate mofetil (Cellcept®) |
Action
Suppress immune response Impair lymphocytes May inhibit collagen cross-linking Suppress immune response Impair lymphocytes |
Most Common Side Effects
Bone marrow damage, hair loss, nausea, diarrhea, bladder inflammation, increased cancer risk, increased infections Tremor, headache, hypertension, nausea, vomiting, kidney & liver injury, infections Bone marrow damage (rare), kidney damage, mouth sores, skin rash, nausea, diarrhea Liver inflammation, nausea, mouth sores, skin rash Tremor, headache, hypertension, chest pain, GI hemorrhage, nausea, vomiting, anemia, fever, infection, sepsis, leg edema |
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Prescription Drugs pilocarpine hydrochloride (Salagen®) |
Action Improves dry mouth symptoms in patients with Sjögren’s |
Most Common Side Effects Dizziness, headache, flushing, urinary frequency, nausea, sweating, hypertension, tachycardia |
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Over-the-Counter Products Dry Mouth: Salivart® Dry Eyes: Artificial tears® |
Action
Saliva substitute, alleviate dry mouth Provide tear-like lubrication |
Most Common Side Effects
No significant side effects observed
Mild stinging, temporary blurred vision |
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Selective Serotonin Reuptake Inhibitors (SSRI's) sertraline (Zoloft®) |
Action Improve symptoms of depression and peripheral pain; Prozac may also improve Raynaud's |
Most Common Side Effects Headache,insomnia, sleepiness, nervousness, diarrhea, nausea, constipation (Paxil® only) |
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Tricyclic Antidepressants amitriptyline (Elavil®) |
Action Improve symptoms of depression, may improve restorative sleep |
Most Common Side Effects Drowsiness, dry mouth, blurred vision, orthostatic hypotension, weight gain, constipation |
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Other Treatments bupropion (Wellbutrin®) nefazodone (Serzone®) trazadone (Desyrel®) |
Action Improve symptoms of depression Improve depression May improve depression-related insomnia |
Most Common Side Effects Headache, insomnia, dry mouth, high blood pressure, nausea
Sedation, nausea, orthostatic hypotension |
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Medications (none proven in controlled trials; hydroxychloroquine sulfate (Plaquenil®) methotrexate (Rheumatrex®, Trexall®)
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Action
Suppress inflammation Unclear
May inhibit collagen cross-linking Decrease skin thickening |
Most Common Side Effects
Liver inflammation, nausea, mouth sores, skin rash
Dizziness, confusion, nausea, vomiting Skin rash, nausea, diarrhea
Bone marrow damage (rare), kidney damage, mouth sores, skin rash, nausea, diarrhea Burning, itching, nausea, skin aging |
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Skin Itching (Pruritis)/Dryness To relieve dry itchy skin, it is necessary to moisturize often. Also, you must protect your skin from harsh detergents, hot water, and the cold winter air, all of which will rob moisture from your skin. You may want to consider using a humidifier in the home during the cold winter heating months to replace much-needed moisture into the air. Remember that not everyone will have the same response to every product. You may have to try a few products until you find one that will work for you. |
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Over-the-Counter Skin Lotions |
Action |
Most Common Side Effects |
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Antihistamines (Prescription) diphenhydramine (Benadryl®) Colchicine |
Action Block histamine response, thereby decreasing itching Reduce inflammation associated with calcinosis |
Most Common Side Effects Drowsiness, dry mouth Diarrhea |
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To learn more about prescription and over-the-counter medication as well as new medications, supplements and herbal remedies not listed in the table above, the U.S. National Library of Medicine National Institutes of Health has developed the "MedlinePlus" web-site, which can be found at:
ww.nlm.nih.gov/medlineplus/druginformation.html
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