Scleroderma (also known as systemic sclerosis) is a chronic autoimmune rheumatic disease. An estimated 240 per million Americans have this disease; most are women between the ages of 30 and 50.
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Hardening of the skin is one of the most visible signs of this disease. The word scleroderma comes from two Greek words: sclera meaning hard, and derma meaning skin.
It is a disease in which dense, thick fibrous tissue takes the place of normal tissue. This happens when your immune system triggers cells to overproduce collagen, a protein that provides strength and elasticity to skin, bone and other connective tissue. The extra collagen is deposited in the skin and organs, which causes hardening and thickening that is similar to scarring.
Scleroderma most often affects the skin. But the disease also can affect many other parts of the body, including the digestive system, lungs, kidneys, heart, blood vessels, muscles and joints.
Prompt and proper diagnosis and treatment may minimize the symptoms of scleroderma and lessen the chance of irreversible damage. We talked with Kristin Highland, MD, a pulmonologist who specializes in scleroderma-associated lung disease, and rheumatologist Soumya Chatterjee, MD, MS, FRCP(UK), FACP, FACR to learn more.
Q: Does scleroderma run in families?
A: It is not uncommon for autoimmunity to run in families, Dr. Highland says. For example, one patient could have multiple sclerosis (MS), another could have scleroderma or lupus and yet another might have thyroid disease.
However, the risk is only slight. There is one exception in that the prevalence of scleroderma in the Choctaw Native Americans who originally settled in Oklahoma is quite increased. It is important to share your family history with your physicians.
Q: What causes shortness of breath for people with scleroderma?
A: There are multiple potential causes of shortness of breath in patients with scleroderma, Dr. Highland says. These include: interstitial lung disease, also called pulmonary fibrosis or scarring of the lungs; pulmonary hypertension, which is high blood pressure in the lungs; left heart disease, such as diastolic dysfunction, systolic dysfunction, valvular heart disease or even coronary artery disease; and muscle weakness and deconditioning.
Q: How can people with scleroderma help ease the fatigue and pain in their joints?
A: Fatigue in scleroderma can be a result of multiple complications, and the management will depend on which of these complications is contributing most, such as anemia, thyroid disease, sleep apnea, or Sjogren’s syndrome, Dr. Chatterjee says. It is important to discuss this with your rheumatologist, who can figure out the cause of your fatigue and help address it.
Joint pain can be a result of inflammatory arthritis, Dr. Chatterjee says. If this is the case, medications are available to control it, and again this should be assessed by the physician before a decision can be made. Joint pain also can be a result of mechanical damage to the joints along with central pain from concomitant fibromyalgia. So the management of joint pain will depend on the cause of it in any patient.
Q: Why do some people with scleroderma report their gut sometimes feel funny?
A: Gastrointestinal involvement can be quite different from patient to patients who have scleroderma, Dr. Chatterjee says. Symptoms can include:
- Poorly controlled acid reflux disease
- Impairment of the muscles of the esophagus, which causes swallowing problems
- Slowed ability of the stomach to move food, also called gastroparesis, which causes bloating and feeling full after a small meal
- Episodic diarrheafrom bacterial overgrowth
- Fecal incontinence, that occurs in some patients due to fibrosis of the anal sphincter