The board-certified physicians at Norton Rheumatology Specialists treat scleroderma in the Louisville area and Southern Indiana. Autoimmune diseases such as scleroderma can affect the whole body. Part of an effective treatment plan means working with specialists in orthopedics, heart and vascular, gastroenterology, and more who are part of Norton Healthcare’s comprehensive system.
Like lupus, scleroderma (literally “hard skin”) is a chronic autoimmune disease where the body’s immune system attacks the body’s tissues. Scleroderma results in the hardening of your body’s connective tissue. Connective tissue is made of many kinds of protein, including collagen, and supports the body’s organs.
In scleroderma, cells start making collagen as if there were an injury that needs repairing. The cells do not turn off as they should and end up making too much collagen. The extra collagen in the tissues can prevent the body’s organs from functioning normally.
Scleroderma Symptoms and CREST Syndrome
Symptoms vary from person to person, depending on factors such as age and the area of the body affected. The collection of symptoms is called CREST syndrome, referring to five main symptoms: calcinosis, Raynaud’s phenomenon, esophageal dismobility, sclerodactyly, telangiectasias.
- Calcinosis: Most people with scleroderma experience a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted.
- Raynaud’s phenomenon: One of the earliest signs of systemic scleroderma is Raynaud’s phenomenon, which causes the small blood vessels in your fingers and toes to contract in response to cold temperatures or emotional distress. When this happens, your fingers or toes may turn blue or feel painful or numb. (When this condition occurs on its own and not as a result of another illness, it is called Raynaud’s disease.)
- Esophageal dysmotility: Scleroderma can cause a variety of digestive symptoms, depending on which part of the digestive tract is affected. If the esophagus is affected, you might have heartburn or difficulty swallowing. If the intestines are affected, you might have cramps, bloating, diarrhea or constipation.
- Sclerodactyly: When collagen deposits in the skin, it gradually hardens and tightens, especially near the joints of the fingers.
- Telangiectasias: Spider veins appear on areas of the face, palms or mucus membranes. These telangiectasias get worse over time.
Medications that help rheumatoid arthritis or lupus typically don’t help scleroderma patients. Your Norton Rheumatology Specialists rheumatologist will look to curb your symptoms and prevent further complications.
- Raynaud’s phenomenon can be treated with calcium channel blockers, or PDE-5 inhibitors — sildenafil and tadalafil — open up narrowed blood vessels and improve circulation. Keeping the body warm, especially extremities like fingers and toes, can help prevent additional damage.
- The heartburn or acid reflux that comes with esophageal dismobility can be treated with antacids and proton-pump inhibitors to ease gastroesophageal reflux disease (GERD).
- Blood pressure medications — ACE inhibitors — can control scleroderma kidney damage if started early.
- Intravenous immunoglobin can help with muscle pain and weakness. Physical and occupational therapy can help preserve flexibility in the joints and skin especially when started early.
- Interstitial lung disease and pulmonary arterial hypertension can be treated with new drugs and new uses of established medications.