Medication Guide for Scleroderma

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1 Medication Guide for Scleroderma 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-thecounter 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 Raynaud's Phenomenon Calcium Channel Blockers nifedipine (Procardia, Adalat ) amlodopine (Norvasc ) isradipine (Dynacirc ) diltiazem (Cardizem, Dilacor XR ) nicardipine (Cardene ) nisoldipine (Sular ) felodipine (Plendil ) Angiotensin II Receptor Antagonists losartan (Cozaar ) valsartan (Diovan ) Relax blood Block constriction of blood Low blood pressure, constipation, nausea, may worsen swallowing difficulty, light-headedness, headache, leg edema, palpitations, irregular heartbeats Diarrhea, dizziness, cough, muscle pain

2 Others prazosin (Minipress ) doxazosin (Cardura ) Relax blood Low blood pressure pentoxifylline (Trental ) Unclear Gastrointestinal Symptoms Gastroesophageal Reflux Disease (GERD) Antacids Gaviscon, Maalox, Mylanta, Rolaids, Neutralize Tums stomach acidity H-2 Blockers cimetidine (Tagamet ) ranitidine (Zantac ) famotidine (Pepcid ) nizatidine (Axid ) Proton Pump Inhibitors omeprazole (Prilosec ) lansoprazole (Prevacid ) esomeprazole (Nexium ) rabeprazole (Aciphex ) pantoprazole (Protonix ) Others sucralfate (Carafate ) Gastrointestinal Symptoms Improve Swallowing GI Stimulants bethanecol (Urecholine ) metoclopramide(reglan ) erythromycin octreotide acetate (Sandostatin ), an injectable medication Inhibit stomach acid secretion Inhibit stomach acid secretion Nausea, dizziness Diarrhea, constipation Mental confusion (cimetidine only), diarrhea, headache, dizziness Diarrhea, headache, dizziness Coat esophagus & Constipation stomach; forms protective barrier Stimulate intestinal Abdominal cramping, diarrhea, muscle dizziness, headache, drowsiness contractions, may (Reglan only) improve heartburn Gastric irritation, nausea Can be helpful in very severe cases for improving the motion of the Low heart rate, cardiac arrhythmias, nausea, diarrhea, pain at injection site, expense

3 Gastrointestinal Symptoms Relieve Constipation Bulking Agents calcium polycarbophil (FiberCon, Fiberall ) psyllium (Metamucil ) Softening Agents docusate calcium (Surfak, Sulfolax ) docusate sodium (Colace ) Others lactulose (Cephulac, Cholac ) polyethylene glycol (MiraLax ) bowel Soften stool Soften stool Make bowels move Abdominal bloating, flatulence Mild abdominal cramping Abdominal cramping, flatulence Nausea, abdominal bloating, cramping, flatulence 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. Broad Spectrum Antibiotics Examples include: tetracycline ampicillin metronidazole (Flagyl ) vancomycin, ciprofloxacin (Cipro ) amoxicillin/clavulanate (Augmentin ) clarithromycin (Biaxin ) azithromycin (Zithromax ) Decrease bacterial GI upset, diarrhea, nausea, and overgrowth vomiting, prolonged use may be complicated by superinfection Joint & Tendon Pain Non-Steroidal Anti-Inflammatory

4 Drugs (NSAIDs) Suppress flurbiprofen (Ansaid ) inflammation fenoprofen (Nalfon ) nabumeton (Relafen ) diclofenac (Voltaren, Cataflam ) diclofenac & misoprostol (Arthrotec ) diclofenac sodium (Voltaren ) etodolac (Lodine ) ibuprofen (Motrin, Advil, Nuprin ) indomethacin (Indocin ) ketoprofen (Orudis ) ketorolac (Toradol ) meloxicam (Mobic ) naproxen (Naprosyn, Anaprox, Aleve ) oxaprozin (Daypro ) piroxicam (Feldene ) suldinac (Clinoril ) COX-2 Inhibitors celecoxib (Celebrex ) Analgesics acetaminophen (Tylenol ) tramadol (Ultram ) Suppress inflammation Relieve pain Narcotics acetaminophen/propoxyphene Relieve severe (Darvocet-N ) pain acetaminophen/hydrocone (Vicodin ) acetaminophen/oxycodone (Percocet ) GI upset, stomach ulcers, impaired kidney function, liver inflammation, confusion Headache, edema, rash Risk of liver & kidney damage Esophagus irritation & sedation Addiction possible, constipation, dizziness, drowsiness, nausea, vomiting oxycodone hydrochloride (OxyContin, OxyIR ) Sedation, low blood pressure, nausea, vomiting Pulmonary Fibrosis/Alveolitis Immunosuppressants

5 (none proven in controlled trials; the following have been used) cyclophosphamide (Cytoxan ) azathioprine (Imuran ) cyclosporine (Neoral, Sandimmune ) mycophenolate mofetil (Cellcept ) Suppress immune response Suppress immune response Impair lymphocytes Impair lymphocytes Bone marrow damage, hair loss, nausea, diarrhea, bladder inflammation, increased cancer risk, increased infection Nausea, pancreatitis, increased infection, increased cancer risk, anemia Tremor, headache, hypertension, nausea, vomiting, kidney & liver injury, infections Tremor, headache, hypertension, chest pain, GI hemorrhage, nausea, vomiting, anemia, fever, infection, sepsis, leg edema 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. Endothelin Receptor Antagonists ambrisentan (Letairis ) bosentan (Tracleer ) Prostaglandin Derivatives epoprostenol (Flolan ) iloprost (Ventavis ) inhalation Edema, and birth defects Potential liver injury, headache, flushing, edema, anemia, male infertility, birth defects 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

6 solution trepostinil (Remodulin ) Calcium Channel Blockers Can be helpful to a small proportion of See Raynaud's patients; see Raynaud s section section Phosphodiesterase Type 5 (PDE5) Inhibitor sildenafil citrate (Revatio ) tadalafil (Adcirca ) Renal Crisis/New Onset Hypertension ACE Inhibitors captopril (Capoten ) Block constriction enalapril (Vasotec ) of blood lisinipril (Prinivil, Zestril ) quinapril (Accupril ) ramipril (Altace ) fosinopril (Monopril ) benazopril (Lotensin ) moexipril (Univasc ) trandolapril (Mavik ) Headache, flushing, pain at infusion site See Raynaud's section headache, dyspepsia, flushing, nosebleeds, insomnia headache, stomach upset, back pain, muscle pain, nasal stuffiness, flushing, pain in arms or legs, dizziness, or vision change Skin rash, dry mouth, taste disturbances, fluid retention, drowsiness, light-headedness, diarrhea, cough If taking corticosteroids, monitor your blood pressure frequently, and report changes to your healthcare provider immediately.

7 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. Skin Fibrosis Immunosuppressants (none proven in controlled trials; the following have been used) cyclophosphamide (Cytoxan ) cyclosporine (Neoral ), Sandimmune ) D-penicillamine (Cuprimine, Depen ) methotrexate (Rheumatrex, Trexall ) mycophenolate mofetil (Cellcept ) Sjögren's Syndrome Prescription Drugs pilocarpine hydrochloride (Salagen ) Suppress immune response Impair lymphocytes May inhibit collagen crosslinking Suppress immune response Impair lymphocytes Improves dry mouth symptoms in patients with Sjögren s 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 Dizziness, headache, flushing, urinary frequency, nausea, sweating, hypertension, tachycardia

8 cevimeline hydrochloride (Evoxac ) Over-the-Counter Products Dry Mouth: Salivart Headache, rhinitis, diarrhea, nausea, sweating, urinary frequency Saliva substitute, No significant side effects observed alleviate dry mouth Biotene Oral Balance Moisturizing Gel Biotene Dry Mouth Gum Biotene Dry Mouth Toothpaste Biotene Dry Mouth Mouthwash Optimoist Salix Lozenges Dry Eyes: Artificial tears Reactive Depression Selective Serotonin Reuptake Inhibitors (SSRI's) sertraline (Zoloft ) paroxetine (Paxil ) fluoxetine (Prozac ) fluvoxamine (Luvox ) Tricyclic Antidepressants amitriptyline (Elavil ) nortriptyline (Pamelor ) imipramine (Tofranil ) clomipramine (Anafranil ) Other Treatments bupropion (Wellbutrin ) venlafaxine (Effexor ) nefazodone (Serzone ) Provide tear-like lubrication Mild stinging, temporary blurred vision Improve symptoms Headache,insomnia, sleepiness, of depression and nervousness, diarrhea, nausea, peripheral pain; constipation (Paxil only) Prozac may also improve Raynaud's Improve symptoms Drowsiness, dry mouth, blurred vision, of depression, may orthostatic hypotension, weight gain, improve restorative constipation sleep Improve symptoms Headache, insomnia, dry mouth, high of depression blood pressure, nausea Improve depression

9 trazadone (Desyrel ) Localized Scleroderma Medications (none proven in controlled trials; the following have been used) hydroxychloroquine sulfate (Plaquenil ) methotrexate (Rheumatrex, Trexall ) prednisone (Deltasone, Cortan ) May improve depression-related insomnia Sedation, nausea, orthostatic hypotension Unclear Suppress immune response Suppress inflammation Seizures, leukopenia, thrombocytopenia Liver inflammation, nausea, mouth sores, skin rash Fluid retention, GI irritation phenytoin (Dilantin ) potassium p-aminobenzoate (POTABA ) Unclear Unclear Dizziness, confusion, nausea, vomiting Skin rash, nausea, diarrhea D-penicillamine (Cuprimine, Depen ) PUVA (phototherapy) May inhibit collagen crosslinking Decrease skin thickening Bone marrow damage (rare), kidney damage, mouth sores, skin rash, nausea, diarrhea Burning, itching, nausea, skin aging 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. Over-the-Counter Skin Lotions KeriCream Lanalor Lubriderm Moisturize skin No significant side effects observed

10 Eucerin Moisturizing creams and lotions Nivea Moisturizing UltraDerm Alpha Keri Bath Oil Penederm alpha hydroxy cream Aveeno bath oil & moisturizer Antihistamines (Prescription) diphenhydramine (Benadryl ) hydroxyzine (Atarax ) Colchicine Block histamine response, thereby decreasing itching Reduce inflammation associated with calcinosis Drowsiness, dry mouth Diarrhea 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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