Progressive systemic sclerosis; Systemic sclerosis; Limited scleroderma; CREST syndrome; Localized scleroderma; Morphea - linear; Raynaud's phenomenon - scleroderma
Scleroderma is a disease that involves the buildup of scar-like tissue in the skin and elsewhere in the body. It also damages the cells that line the walls of the small arteries.
Scleroderma is a type of autoimmune disorder. In this condition, the immune system mistakenly attacks and damages healthy body tissue.
The cause of scleroderma is unknown. A buildup of a substance called collagen in the skin and other organs leads to the symptoms of the disease.
The disease most often affects people 30 to 50 years old. Women get scleroderma more often than men. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.
Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis. These cases are referred to as undifferentiated connective tissue disease or overlap syndrome.
Some types of scleroderma affect only the skin, while others affect the whole body.
Localized scleroderma, (also called morphea) -- Often affects only the skin on the chest, abdomen, or limb but not usually on the hands and face. Morphea develops slowly, and rarely spreads in the body or causes serious problems such as internal organ damage.
Systemic scleroderma, or sclerosis -- May affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types, limited disease (CREST syndrome) and diffuse disease.
Skin signs of scleroderma may include:
- Fingers or toes that turn blue or white in response to cold temperatures (Raynaud phenomenon)
- Stiffness and tightness of skin of fingers, hands, forearm, and face
- Hair loss
- Skin that is darker or lighter than normal
- Small white lumps of calcium beneath the skin that sometimes ooze a white substance that looks like toothpaste
- Sores (ulcers) on the fingertips or toes
- Tight and mask-like skin on the face
- Telangiectasias, which are small, widened blood vessels visible beneath the surface on the face or at the edge of fingernails
Bone and muscle symptoms may include:
- Joint pain, stiffness, and swelling, resulting in loss of motion. The hands are often involved due to fibrosis around tissue and tendons.
- Numbness and pain in the feet.
Breathing problems may result from scarring in the lungs and can include:
- Dry cough
- Shortness of breath
- Increased risk for lung cancer
Digestive tract problems may include:
Heart problems may include:
- Abnormal heart rhythm
- Fluid around the heart
- Fibrosis in heart muscle, decreasing heart function
Kidney and genitourinary problems may include:
- Development of kidney failure
- Erectile dysfunction in men
- Vaginal dryness in women
Exams and Tests
The health care provider will do a complete physical exam. The exam may show:
- Tight, thick skin on the fingers, the face or elsewhere.
- The skin at the edge of the fingernails may be looked at with a lighted magnifying glass for abnormalities of the small blood vessels.
- The lungs, heart and abdomen will be examined for abnormalities.
Your blood pressure will be checked. Scleroderma can cause small blood vessels in the kidneys to become narrowed. Problems with your kidneys can lead to high blood pressure and decrease function of the kidney.
Blood and urine tests may include:
Other tests may include:
There is no specific treatment for scleroderma. Your provider will assess the extent of disease in the lungs, kidneys, heart, and gastrointestinal tract.
You will be prescribed medicines and other treatments to control your symptoms and prevent complications.
Medicines used to treat scleroderma include:
- Corticosteroids such as prednisone -- Doses above 10 mg per day are not recommended because they can increase the risk for kidney failure.
- Drugs that suppress the immune system, such as methotrexate or mycophenolate.
- Nonsteroidal anti-inflammatory drugs (NSAIDs) for short periods.
- Hydroxychloroquine to treat arthritis.
- Blood pressure medicines, such as ACE inhibitors for high blood pressure -- Blood pressure should be watched regularly and closely, especially in people who have rapidly progressive skin disease. A sudden rise of blood pressure could indicate the start of kidney failure, which would require emergency treatment.
Other treatments for specific symptoms may include:
- Treatments to improve Raynaud phenomenon -- These include medicines called long acting calcium channel blockers (nifedipine, amlodipine), thick gloves to keep the hands warm, and not smoking.
- Medicines for heartburn or swallowing problems, such as omeprazole.
- Laser light therapy to decrease telangectesias.
- Medicines to improve blood flow in the lungs, such as bosentan and sildenafil.
Treatment often involves physical therapy as well.
Some people can benefit from attending a support group for people with scleroderma.
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.
People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to.
- Heart failure
- Scarring of the lungs, called pulmonary fibrosis
- High blood pressure in the lungs (pulmonary hypertension)
- Kidney failure (scleroderma renal crisis)
- Problems absorbing nutrients from food
When to Contact a Medical Professional
Call your provider if you develop Raynaud phenomenon, progressive thickening of the skin, or trouble swallowing.
Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-1339. PMID: 27941129 www.ncbi.nlm.nih.gov/pubmed/27941129.
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