Treatments & Medications
Scleroderma can cause different symptoms in each person with the disease. Your treatment plan is designed just for your disease and individual symptoms.
There are medications your rheumatologist can prescribe to help you manage localized scleroderma, usually skin symptoms and Raynaud’s phenomenon.
Other medications treat systemic disease, or scleroderma that affects not just your skin, but your organs and connective tissues in your body. Systemic medications work to control your disease activity, or inflammation caused by your immune system, to help control serious conditions like interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH).
Medications for scleroderma may also treat symptoms that affect your quality of life and independence, like pain or stiffness in your joints or muscles, or GI problems like heartburn or acid reflux (GERD).
Localized Skin Involvement: Raynaud’s Medications
Raynaud’s phenomenon is a common condition in people with scleroderma. Blood flow to your fingers is greatly reduced because the blood vessels become narrowed or constricted.
Sometimes, your toes, feet, ankles or other body parts are affected too. Your fingers may change color, turning white or red when you’re exposed to cold or have high amounts of stress. Some people have pain or swelling in their hands due to Raynaud’s. In more severe cases, people may develop sores or ulcerations, or even gangrene, in their extremities.
Blood pressure medications open constricted blood vessels. Normal blood flow is restored to your fingers and toes, feet or ankles. There are other, newer prescription medications that can treat ulcerations from more severe Raynaud’s, and topical creams to treat mild symptoms.
Don’t take OTC meds that contain pseudoephedrine, because it can trigger Raynaud’s symptoms. Pseudoephedrine is available at pharmacies to treat colds or nasal congestion. It constricts blood vessels. Ask your doctor or pharmacist to suggest other medications for colds or congestion.
Blood pressure medicine is prescribed to manage Raynaud’s phenomenon. These medications open constricted blood vessels. They can restore feeling, warmth, normal color and blood circulation in your hands or feet:
Calcium channel blockers: Nifedipine or amlodipine
Possible side effects: Because calcium channel blockers open your blood vessels, your blood pressure drops. So, you may have swelling in your lower extremities, like your feet or ankles. Let your doctor know if you have this swelling, also called edema.
Angiotensin receptor blockers (ARBs): Losartan or valsartan
Possible side effects: Dizziness, elevated potassium in your blood, swelling (edema)
Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynaud’s phenomenon symptoms, such as ulcerations on the fingers. They open blood vessels and restore blood flow to extremities. If these ulcerations are not controlled, they can worsen. You could develop gangrene and risk losing your fingers. PED-5 inhibitors can restore blood flow and treat the painful ulcerations on your skin.
PED-5 inhibitors to treat Raynaud’s: Sildenafil or tadalafil
Possible side effects: Low blood pressure, headaches, dizziness, fatigue
Topical nitroglycerine cream or paste can be used to treat mild, localized Raynaud’s symptoms like a skin ulcer. Rub the cream on the skin of your fingers, and then slip on some gloves to keep your hands warm and help your skin absorb the topical cream.
Other Raynaud’s treatments and lifestyle tips:
Botulinum toxin injections may be used to treat isolated ulcers on your fingers, but trial results for this treatment are mixed.
Sympathectomy is a minor surgical procedure where tiny nerves connected to arteries in your fingers are cut. Sympathectomy may only be done after other medications haven’t worked.
Lifestyle changes may help you manage or prevent Raynaud’s symptoms too:
- Keep your fingers, hands, feet and toes warm, especially during winter or if you’re in a cold indoor space.
- Wear a hat in winter: body heat escapes through your head.
- Find ways to manage stress. Stress can trigger blood vessel spasms and Raynaud’s symptom flare-ups. Exercises like walking, yoga or tai chi can be helpful ways to ease stress.
- Avoid smoking and secondhand smoke.
- Cut back on your caffeine or stimulant medicines (like cold medicine).
Pain Medications
Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. Talk with your rheumatologist about non-drug treatments to help manage pain too.
Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called “anti-inflammatories.” These are common pain medications. They’re available OTC in both brand name products and generics (store brands). NSAIDs can treat occasional flare-ups of joint pain. NSAIDs include ibuprofen, naproxen sodium and diclofenac sodium.
Possible side effects: NSAIDs can irritate the lining of your esophagus and stomach, causing stomach pain or, more severely, ulcers. NSAIDs may also affect your kidneys, so they may not be an option for everyone with scleroderma.
Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling. The most common steroid is prednisone. It’s taken as a pill. Typically, if you need a steroid, they’ll prescribe a short-term course of the lowest dose you need, and each pill will have a decreased amount of steroid so you “taper” your intake of the drug. Steroids can cause severe side effects if taken in higher doses or for too long, so your doctor will only prescribe the lowest dose and the shortest course you need.
Possible side effects: Weight gain, mood changes, dizziness, headache, thinning skin and bruising, puffy face, heartburn, increases in blood sugar, muscle weakness, sleep disruption or insomnia, osteoporosis (thinning bones), others. If you’ve tested positive for the RNA polymerase-3 antibody, steroids could cause scleroderma renal crisis, so you may need to avoid steroids.
Opioids are also called narcotics. They include prescription medications used to relieve pain only. Opioids include hydrocodone and oxycodone. They’re taken as pills.
Some people with scleroderma pain may need to take opioids occasionally, but they can have severe side effects and carry a risk of dependence. Your doctor may prescribe opioids for the short term and then switch to other medications, like gabapentin or duloxetine, to treat your pain. Opioids often cause constipation. Many people with scleroderma already have constipation, so these pain medications can worsen it.
Possible side effects: Constipation, dizziness, nausea, vomiting, dependence/addiction, respiratory depression
Medications for Skin Involvement
Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. They’re also called immunosuppressants. By lowering your overactive immune system response, these medications can help improve skin fibrosis and your skin-related symptoms.
Immunosuppressants and infection risk: All immunosuppressant medications lower your immune system, so they make it harder for you to fight off all types of infections. Talk with your rheumatologist about this risk, how to protect yourself from infections while taking an immunosuppressant, and what to do if you think you have an infection.
Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. It comes in two forms: mycophenolate mofetil and mycophenolate sodium. Mycophenolate is available as a tablet, capsule, delayed-release tablet, or liquid that you swallow.
Possible side effects: Diarrhea that usually improves over time, low blood cell counts, infections. Mycophenolate could elevate your liver enzymes, so your doctor will test you for hepatitis before you start taking it. They will also test you for tuberculosis, a serious lung infection. Avoid pregnancy while taking mycophenolate, or let your doctor know if you think you may be pregnant. Mycophenolate may also increase your risk of certain cancers, including lymphoma and skin cancer. Let your doctor know if you or anyone in your family has had cancer.
Cyclophosphamide is another immunosuppressant medication that’s been shown to improve skin fibrosis in scleroderma. It’s available as both a pill and an infused drug that you take through an IV (intravenous drip). Cyclophosphamide may be an option for people with more severe disease complications, but it can cause toxicity, so mycophenolate is often the preferred option.
Possible side effects: Increases risk of infection, kidney or bladder problems, fertility problems. Before and during treatment with cyclophosphamide, your doctor will give you kidney function and urine screening tests. Cyclophosphamide can irritate the lining of your bladder. If you notice blood in your urine while taking cyclophosphamide, let your doctor know immediately.
Methotrexate is another immunosuppressant that’s been used for decades to treat inflammatory diseases, including rheumatoid arthritis. It may be prescribed to treat scleroderma skin and joint involvement. Methotrexate is taken by mouth as a pill.
Possible side effects: Nausea, vomiting, mouth sores, hair thinning, infections. Your doctor will prescribe folic acid along with your methotrexate to help prevent mouth sores. Since methotrexate is metabolized in your liver, you’ll need a blood test to check your liver function every three months. Methotrexate could cause serious birth defects, so don’t use this drug if you’re pregnant or think you may be pregnant. Avoid pregnancy or conception while taking methotrexate.
Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. As the name suggests, it’s taken as an IV drip into your vein. You’ll go to a special infusion center or clinic to get your IVIG treatments.
Possible side effects: Headache, dizziness, fatigue.
Medications for GI Symptoms
People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux. These symptoms can be very painful, keep you awake at night, and ruin your quality of life. If reflux becomes severe, it can damage your esophagus. Medications may help relieve GI symptoms of scleroderma, and help control acid reflux to protect your GI tract.
Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. Proton pump inhibitors include omeprazole, pantoprazole and lansoprazole.
H2-receptor antagonists or H2 blockers are also available in both OTC and prescription strength products. They also control stomach acid to help manage reflux and GI symptoms. H2 blockers include famotidine, cimetidine and nizatidine. Another H2 blocker, ranitidine, is currently off the market, but may return at some point.
Possible side effects: Decreased absorption of some vitamins and minerals. Some H2 blockers also cause headaches.
Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. That’s because your GI tract’s movement is affected by your disease.
Diarrhea: OTC medications that bulk up your stool can ease diarrhea, such as psyllium. You may also need anti-diarrheal medication, such as loperamide. Ask your doctor or nurse if it’s OK to take these medicines occasionally.
Constipation: Fiber supplements may help you have more regular bowel movements. OTC laxatives are an option to treat occasional constipation, but talk to your doctor or nurse about how often to take laxatives and which products to use. A newer prescription drug for irritable bowel syndrome called linaclotide may be an option for scleroderma-related constipation too.
Systemic Medications: Scleroderma-Associated ILD
Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. Biologics target specific “inflammatory” proteins made by your immune system that are involved in scleroderma lung fibrosis. By blocking or suppressing these inflammatory proteins, your medication can help control lung fibrosis, delay progression of lung disease, and improve your symptoms.
Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. Nintedanib is a pill you take twice a day. It’s called a tyrosine kinase inhibitor.
Possible side effects: diarrhea, nausea, abdominal pain, vomiting, liver enzyme elevation, decreased appetite, headache, weight loss, hypertension (high blood pressure) and infections, including pneumonia. Nintedanib may harm a fetus, so it shouldn’t be used during pregnancy or if you think you’re pregnant. Nintedanib also may be risky for anyone with elevated liver enzymes, drug-induced liver injury, or gastrointestinal disorders.
Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. It’s an IL-6 inhibitor, meaning it targets and blocks an inflammatory protein or “cytokine” called interleukin-6 (IL-6). Tocilizumab is taken as either a subcutaneous injection you give yourself at home, or as an infusion (IV) at a clinic.
Possible side effects: Increased risk of infections such as tuberculosis or fungal infections, reduced platelet counts, elevated liver enzymes, allergic reactions to IV infusions like chills or fever (rare), increased cholesterol levels, bowel perforations (rare). You’ll be tested for tuberculosis before you start this drug. You may need to have your cholesterol levels monitored, and prescribed medication if your cholesterol is too high. If you’ve had diverticulitis in the past, or if you notice bloody stool or abdominal pain, let your doctor know immediately.
Pulmonary Hypertension and Kidney Complications
People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. Medications that lower blood pressure may be prescribed to treat these complications.
Pulmonary hypertension affects as many as 40% of people with scleroderma. It can happen in both limited and systemic scleroderma.
Pulmonary hypertension is high blood pressure in the vessels in your lungs. You may notice shortness of breath, especially during any type of activity, as well as fainting and discomfort in your chest.
In pulmonary hypertension, blood vessels may become narrowed or constricted. Medications can help open up your blood vessels to increase blood flow in your lungs, and to reduce stress on your heart when it pumps blood to deliver oxygen to your lungs. These treatments can lower that blood pressure in your lungs and relieve your symptoms.
Pulmonary hypertension medications include:
- Ambrisentan, bosentan and macitentan
- Sildenafil and tadalafil (also used to treat Raynaud’s, above)
- Epoprostenol, iloprost, tresprostinil
- Selexipag and riociguat, which are newer medications currently being studied as possible treatments for pulmonary hypertension
Kidney complications with scleroderma can be very serious, even life-threatening. Usually, only people with diffuse scleroderma develop renal crisis or other serious kidney complications. It’s rare in people with limited scleroderma.
If you develop scleroderma renal crisis, blood vessels in your kidneys spasm and your blood pressure can become very high. You’ll be admitted to the hospital for aggressive treatment to lower your blood pressure, prevent kidney injury, and save your organs.
Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital. Eventually, your doctor will switch you to another blood pressure medicine, such as other ACE inhibitors like lisinopril or enalapril.
NSAIDs and steroids used to treat joint pain flare-ups may increase blood pressure as a side effect, so if you have scleroderma kidney complications, you may need to try other pain treatments.
No two scleroderma journeys are the same, but there are common experiences along the way. No matter where you, your child, or a loved one are in your journey, or the type of scleroderma, the National Scleroderma Foundation can help you find your best path.