There is currently no cure for the hallmark symptom of scleroderma — skin thickening and hardening. In fact, no cure exists for the autoimmune disease at all, but different therapies are available to treat various conditions associated with scleroderma. Known as palliative therapies, these treatments help manage the disease’s wide range of conditions and their related symptoms, and so maintain a patient’s quality of life.

Below are a number of these conditions, and their respective therapies.

Therapies for Raynaud’s phenomenon

Raynaud’s phenomenon is usually treated with the same therapies used for Raynaud’s disease, which work toward improving blood flow and blood pressure.

Calcium channel blockers affect calcium movement in cells of the heart and blood.  Common medicines include:

  • nifedipine (Procardia, Adalat)
  • amlodipine (Norvasc)
  • diltiazem (Dilacor XR, Cardizem, Tiazac)
  • verapamil (Calan, Verelan, Isoptin)
  • felodipine (Plendil)
  • nisoldipine (Sular)
  • bepridil (Vascor)
  • isradipine (DynaCirc)
  • nicardipine (Cardene)
  • nisoldipine (Sular)
  • prazosin (Minipress)

The disease can also be treated using angiotensin II receptor antagonists, which block a certain chemical that leads to narrow blood vessels. Common therapies include:

  • losatan (Cozaar)
  • valsartan (Diovan)

Angiotensin converting enzyme (ACE) inhibitors offer another treatment option for Raynaud’s because they block an enzyme from contributing to narrow vessels. Common treatments include:

  • captopril (Capoten)
  • benazepril (Lotensin)
  • lisinopril (Prinivil, Zestril)
  • ramipril (Altace)
  • enalapril (Vasotec)

PDE-5 inhibitors can be prescribed to improve blood circulation by opening up narrowed blood vessels. This action reduces high blood pressure, which reduces the heart’s workload. Common therapies include:

  • sildenafil citrate (Viagra)
  • tadalafil (Cialis)

An important non-pharmacological approach to Raynaud’s phenomenon is keeping the body warm — especially fingers and toes — and avoiding stress triggers. Calm, peaceful exercises and meditation can help reduce stress.

Therapies for heartburn

Heartburn (acid reflux) can be treated with antacids. Many are sold over-the-counter. Common brand names include:

  • Maalox
  • Mylanta
  • Phillips (Milk of Magnesia)
  • Gaviscon
  • Rolaids
  • Tums

Antacids relieve heartburn, sour stomach, and acid indigestion by neutralizing excess stomach acid.

Proton-pump inhibitors and H2-blockers decrease the production of acid which minimizes reflux and helps prevent esophageal ulcers.

Common proton-pump inhibitors include:

  • omeprazole (Prilosec; Prilosec OTC is available over-the-counter; while Zegerid is an immediate-release form of omeprazole)
  • lansoprazole (Prevacid)
  • esomeprazole (Nexium)
  • rabeprazole (Aciphex)
  • pantoprazole (Protonix)

Common H2-blockers include:

  • nizatidine (Axid)
  • famotidine (Pepcid)
  • cimetidine (Tagamet)
  • ranitidine (Zantac)

Therapies for kidney disease

Kidney disease in scleroderma is treated using angiotensin converting enzyme (ACE) inhibitors because these block the formation of an enzyme that narrows blood vessels. The desired effect is relaxed blood vessels and lowered blood pressure. Common therapies are:

  • captopril (Capoten)
  • benazepril (Lotensin)
  • lisinopril (Prinivil, Zestril)
  • ramipril (Altace)
  • enalapril (Vasotec)

Therapies for muscle weakness, joint pain

Muscle weakness and joint pain are usually treated with nonsteroidal anti-inflammatory (NSAIDs) drugs. Anti-inflammatory therapies help relieve inflammation, swelling, stiffness, and joint pain caused by arthritis. Common medicines include:

  • aspirin (Bayer, St. Joseph’s)
  • ibuprofen (Advil, Motrin)
  • naproxen (Naprosyn, Aleve)
  • nabumeton (Relafen)
  • flurbiprofen (Ansaid)
  • fenoprofen (Nalfon)
  • diclofenac (Voltaren, Cataflam)
  • meloxicam (Mobic)
  • diclofenac and misoprostol (Arthrotec)
  • diclofenac sodium (Voltaren)
  • etodolac (Lodine)
  • indomethacin (Indocin)
  • ketoprofen (Orudis)
  • ketorolac (Toradol)
  • oxaprozin (Daypro)
  • piroxicam (Feldene)
  • suldinac (Clinoril)

Low dose glucocorticoids (prednisone) are also used to treat inflammation because they reduce swelling, redness, itching, and allergic reactions. Intravenous immunoglobulin or immunosuppressive medications may also be used for muscle weakness and joint pain. Joint and skin flexibility can be maintained with physical and occupational therapy.

Therapies for pulmonary arterial hypertension in scleroderma

Pulmonary arterial hypertension (PAH) is characterized by high blood pressure in the main artery that carries blood from the right side of the heart to the lungs. When the lung’s smaller arteries become resistant to blood flow, the right ventricle is forced to work harder to pump blood through the lungs. PAH can be treated with prostaglandin derivatives. Common therapies include:

  • epoprostenol (Flolan) and treprostinil (Remodulin) relax blood vessels and increase the blood flow to the lungs
  • iloprost (Ventavis), an inhalable form of prostacyclin, is introduced directly into the lungs which reduces side effects to the rest of the body

Endothelin receptor antagonists may also be prescribed to prevent the small protein endothelin-1 from binding to its receptors. This blockage leads to increased blood supply to the lungs and less need for the heart to work overtime. Common therapies are:

  • bosentan (Tracleer)
  • ambrisentan (Letairis)
  • macitentan (Opsumit)
  • riociguat (Adempas)

PDE5 inhibitors build up a signaling molecule called cGMP which relaxes the muscles, increasing the blood flow in the lungs and decreasing hypertension. Common PDE5 inhibitors include:

  • sildenafil citrate (Viagra, Revatio)
  • tadalafil (Cialis, Adcirca)

Therapies for interstitial lung disease in scleroderma

People with scleroderma may also develop interstitial lung disease, a group of lung disorders in which the lung tissues become inflamed and damaged — such as pulmonary fibrosis. There are different classes of medicines to approach treating these diseases.

The anti-fibrotic agent pirfenidone (Esbriet) inhibits the synthesis of TGF-beta (a chemical mediator with a key role in fibrosis) and TNF-alpha (a cytokine active in inflammation).

Nintedanib (OFEV), a tyrosine kinase inhibitor, targets growth factor receptors that are involved in the mechanisms of fibrosis.

Finally, immunosuppressants work by weakening the body’s immune system so that it will not attack the body in the case of autoimmune disease, and alkylating agents slow or stop cancer growth. Common therapies include:

  • cyclosporine (Neoral, Sandimmune)
  • mycophenolate mofetil (CellCept)
  • azathioprine (Imuran)
  • methotrexate (Rheumatrex, Trexall)
  • D-penicillamine (Cuprimine, Depen)
  • Cyclophosphamide (Cytoxan, Neosar)

Scleroderma News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

 

  1. http://www.scleroderma.org/site/PageNavigator/patients_whatis.html#.WCrw-HetHBI
  2. http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma#sthash.cc9MWRd1.dpuf
  3. http://www.srfcure.org/for-patients/current-treatments
  4. https://medlineplus.gov/ency/article/000128.htm