Scleroderma

Signs and Symptoms

Symptoms of scleroderma may include the following:

Localized scleroderma

Morphea scleroderma: Oval patches of thick skin that are white in the middle and purple around the edges. They usually appear on the chest, back, and stomach, but can also be on arms and legs.

Linear scleroderma: Streaks of hardened skin that appear on arms, legs, or forehead.

Systemic scleroderma

Diffuse cutaneous systemic sclerosis: hardening of skin on fingers, hands, arms, legs, face, neck, and trunk. This type of scleroderma usually affects both sides of the body, meaning if you have problems with your left arm, you'll have problems with your right arm, too. It also can affect internal organs, including heart, lungs, kidneys, and esophagus.

Limited cutaneous systemic sclerosis: Affects skin on fingers, lower arms and legs, face, and neck. People with this type of scleroderma often have CREST syndrome. The term CREST stands for:

• Calcinosis, painful calcium deposits under the skin
• Raynaud's phenomenon, sensitivity to cold in the hands and feet
• Esophageal dysfunction, problems with swallowing caused by internal scarring
• Sclerodactyly, tightening of the skin on the fingers or toes
• Telangiectasia, swollen blood vessels on the hands, palms, forearms, face, and lips

Sine scleroderma: Affects internal organs, but not skin

Seizures and headaches

Causes

Doctors believe scleroderma is caused by the immune system mistakenly attacking the body's own tissues. The immune system attack causes inflammation and triggers the body to make too much collagen. Excess collagen causes the skin, and sometimes the internal organs, to become hard and tight. Researchers are not sure what triggers this autoimmune response. Both genetics and environment may play a role.

Risk Factors

These factors may increase the risk of scleroderma:

• Gender. About 3 times more women than men get scleroderma.
• Age. Scleroderma is most common among people between the ages of 20 to 50. It is relatively rare in children.
• Race and ethnicity. Young women of African ancestry and native Americans of the Choctaw tribe of Oklahoma have high rates of the disease.

Diagnosis

It is not always easy to diagnose scleroderma. You may need to see both a rheumatologist (arthritis specialist) and a dermatologist (skin specialist). The doctor will do a physical examination and feel your skin to check for thickened and hardened areas. The doctor may also press affected tendons and joints and do the following:

• Blood tests, to look for higher levels of antibodies made by the immune system.
• Skin biopsy, to uncover skin problems.
• Chest X ray or pulmonary function test, to assess lung damage.
• MRI or CT scan, to determine whether there is damage to the muscles and internal organs.

Many early scleroderma symptoms are like those of other connective-tissue diseases, such as rheumatoid arthritis, lupus, and polymyositis. When someone has more than one of these diseases, it is called mixed connective-tissue disease.

Preventive Care

Although no one knows how to prevent scleroderma, you can take steps to avoid getting infections when you have scleroderma. Your doctor may recommend:

• Pneumonia (pneumococcal) vaccine
• Annual flu vaccine

Treatment

There is no cure for scleroderma. Medications can treat symptoms and prevent complications. Making changes to your lifestyle and diet can make living with the disease easier.

Lifestyle

These simple steps may help improve quality of life:

• Eat small, frequent meals to reduce heartburn or gas.
• Exercise to help keep skin and joints flexible.
• DO NOT smoke (nicotine makes scleroderma worse).
• Avoid exposure to cold and stress, which can affect circulation.
• Use soothing skin creams to reduce pain, swelling, and stiffness.

Medications

Doctors often treat localized scleroderma with moisturizers or steroid creams. Oral medications, such as minocycline (Minocin or Dynacin), may also be used to stop localized scleroderma from getting worse if it involves a large area of the body, such as an entire arm or leg.

Systemic scleroderma may be treated with medications that improve circulation, reduce heartburn, preserve kidney function, and control high blood pressure. Some medications a doctor may prescribe for scleroderma include:

Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs to relieve joint pain and inflammation.

Medications to promote better circulation: can help prevent high blood pressure and reduce symptoms of scleroderma. They include:

• Calcium channel blockers (CCBs)
• Angiotensin II receptor blockers
• Angiotensin-converting enzyme (ACE) inhibitors
• Alpha blockers
• Aspirin

Disease-modifying antirheumatic drugs (DMARDs): DMARDs slow the progression of the disease. They include:

• Hydroxychloroquinine (Plaquenil)
• Methotrexate (Rheumatrex)
• Sulfasalazine (Azulfidine)

Immunosuppressants: Suppress an overactive immune system. These drugs can have serious side effects including kidney damage and increased risk of infection. They include:

• Azathioprine (Imuran)
• Cyclophosphamide (Cytoxan)
• Cyclosporine (Neoral)

Antacids: To reduce heartburn when there is damage to the esophagus.

Surgery and Other Procedures

When symptoms of scleroderma become very severe, doctors may recommend the following procedures:

• Surgery to fix damage to the stomach or intestinal walls
• Amputation of severely diseased and infected fingers or toes
• Kidney, heart, or lung transplants, in rare cases

Nutrition and Dietary Supplements

People with scleroderma may not get enough vitamins and minerals in their diet, especially if there is damage to their digestive system. Your doctor may suggest you take a supplement. Always tell your doctor about the herbs and supplements you are using or considering using.

These general nutritional tips are good for your overall health, especially if you have a long-lasting disease:

• Eat antioxidant-rich foods, including fruits (such as blueberries, cherries, and tomatoes), and vegetables (such as squash and bell peppers).
• Avoid refined foods, such as white breads, pastas, and especially sugar.
• Eat fewer red meats and more lean meats, cold-water fish, tofu (soy, if no allergy), or beans for protein.
• Use healthy oils, such as olive oil or vegetable oil.
• Reduce or eliminate trans-fatty acids, found in commercially-baked goods such as cookies, crackers, cakes, French fries, onion rings, donuts, and processed foods.
• Avoid caffeine, alcohol, and tobacco.
• Drink 6 to 8 glasses of filtered water daily.
• Exercise at least 30 minutes daily, 5 days a week.

Your doctor may recommend taking a multivitamin daily that has the antioxidant vitamins A, C, E, the B-complex vitamins, and trace minerals, such as magnesium, calcium, zinc, and selenium.

These supplements may help reduce some symptoms:

• Omega-3 fatty acids, such as fish oil, 1 to 2 capsules or 1 to 3 tbsp. of oil, 1 to 3 times daily. Omega-3 fatty acids help improve blood flow, and a few studies suggest they may reduce symptoms of Raynaud's phenomenon and help people better tolerate cold temperatures. Cold-water fish, such as salmon or halibut, are good sources. Omega-3 supplements may increase your risk of bleeding. If you take blood thinners, such as warfarin (Coumadin), clopidogrel (Plavix), or aspirin, ask your doctor before taking omega-3 supplements.
• Bromelain. Although it is not a specific treatment for scleroderma, bromelain helps reduce pain and inflammation. It is often combined with turmeric. Bromelain can interact with a number of medications. It may increase the risk of bleeding, so if you take blood thinners, such as warfarin (Coumadin), clopidogrel (Plavix), or aspirin, ask your doctor before taking bromelain.
• Probiotic supplement (containing Lactobacillus acidophilus or bifidobacterium), 5 to 10 billion CFUs (colony forming units) a day. These "friendly" bacteria help maintain gastrointestinal health. One study found that probiotics helped reduce bloating in people with scleroderma that affected their digestive systems. Be sure to ask your doctor before taking probiotics, because some researchers think they could cause problems when you have an autoimmune disease, or a weakened immune system.

Herbs

Herbs may strengthen and tone the body's systems. As with any therapy, you should work with your health care provider before starting treatment.

Few studies have examined the use of herbs to treat scleroderma. Ask your doctor before taking herbs or supplements.

• Turmeric (Curcuma longa). Lab studies show that turmeric reduces inflammation. It may also help relieve pain, although more studies are needed to tell whether it works for scleroderma. It is often combined with bromelain. Turmeric may increase the risk of bleeding. If you take blood thinners, such as warfarin (Coumadin), clopidogrel (Plavix), or aspirin, ask your doctor before taking turmeric.
• Gotu kola (Centella asiatica), for blood vessel health and circulation. Some purified extracts of gotu kola seem to reduce symptoms of scleroderma. More research is needed. Gotu kola may interact with sedative medications, and may affect the liver. Ask your doctor before taking gotu kola.

Acupuncture

A few studies suggest that acupuncture may improve blood flow in the hands and fingers, help heal fingertip ulcers, and may reduce the formation of fibrous tissue. It may also relieve pain.

Massage and Physical Therapy

Research suggests that massage may help improve circulation. More research is needed to know whether massage works for scleroderma.

Mind-Body Medicine

Biofeedback may help some people with scleroderma better control the temperature in their hands and feet, although studies are mixed. Other mind-body techniques such as counseling, meditation, and emotional freedom technique (EFT) may also help.

Other Considerations

Prognosis and Complications

In some people, symptoms develop quickly for the first few years and continue to get worse. However, in most people, the disease gets worse slowly, over time.

People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to:

• Muscle disorders
• Cancer
• Heart failure
• Scarring of the lungs, called pulmonary fibrosis
• High blood pressure in the lungs (pulmonary hypertension)
• Kidney failure
• Problems absorbing nutrients from food

Supporting Research

Amaral TN, Peres FA, Lapa AT, Marques-Neto JF; Appenzeller S. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum. 2013;43(3):335-47.

Baur JA, Sinclair DA. Therapeutic potential of resveratrol: the in vivo evidence. Nat Rev Drug Discov. 2006;5(6):493-506.

Fauci AS, Braunwald E, Hauser SL, et al, eds. Harrison's Principles of Internal Medicine. 17th ed. New York, NY: McGraw-Hill; 2008.

Frech TM, Khanna D, Maranian P, Frech EJ, Sawitzke AD, Murtaugh MA. Probiotics for the treatment of systemic sclerosis-associated gastrointestinal bloating/ distention. Clin Exp Rheumatol. 2011 Mar-Apr;29(2 Suppl 65):S22-5.

Hale LP, Greer PK, Trinh CT, James CL. Proteinase activity and stability of natural bromelain preparations. Int Immunopharmacol. 2005;5(4):783-93.

Hunnicutt SE, Grady J, McNearney TA. Complementary and alternative medicine use was associated with higher perceived physical and mental functioning in early systemic sclerosis. Explore (NY). 2008 Jul-Aug;4(4):259-63.

Paik JJ, Mammen AL, Wigley FM, Gelber AC. Myopathy in scleroderma, its identification, prevalence, and treatment: lessons learned from cohort studies. Curr Opin Rheumatol. 2014;26(2):124-30.

Shah AA, Wigley FM. My approach to the treatment of scleroderma. Mayo Clin Proc. 2013;88(4):377-93.

Simopoulos AP. Omega-3 fatty acids in inflammation and autoimmune diseases. J Am Coll Nutr. 2002;21(6):495-505.

Wang HK. The therapeutic potential of flavonoids. Expert Opin Investig Drugs. 2000;9(9):2103-19.

Wollina U, Abdel-Naser MB, Mani R. A review of the microcirculation in skin in patients with chronic venous insufficiency: the problem and the evidence available for therapeutic options. Int J Low Extrem Wounds. 2006;5(3):169-80.

Yoon JH, Baek SJ. Molecular targets of dietary polyphenols with anti-inflammatory properties. Yonsei Med J. 2005;46(5):585-96.