For reasons we don’t yet know, women are far more likely to develop scleroderma and other autoimmune disorders than men. Yet many women may be unfamiliar with this disease or any of the over 100 autoimmune disorders because individually their numbers are small. However, taken together, a woman’s chance of developing an autoimmune disease is greater than cancer or heart disease.
This is why we support Scleroderma Awareness Month by sharing important information about this disorder.
What is Scleroderma?
Scleroderma is a chronic autoimmune rheumatic disease affecting connective tissues throughout the body. It stems from an over-production of collagen that creates hardening (sclerosis) of the skin (derma) and internal organs such as the lungs, kidneys, heart and gastrointestinal tract.
Localized scleroderma is often seen more in children. Systemic scleroderma is more common in adults. Overall, female patients outnumber male patients about 4-to-1.
The most common life-threatening effects on the body involve the lungs. Patients with scleroderma are at increased risk for developing pulmonary hypertension (PH), also known as high blood pressure in the blood vessels of the lungs. This reduces the blood oxygen level, which in turn, may cause an increase in blood pressure in the pulmonary arteries.
Scleroderma can also cause high blood pressure by narrowing the pulmonary arteries, a condition called pulmonary arterial hypertension (PAH). When the blood pressure inside the pulmonary vessels is high, the right side of the heart has to pump harder to move blood into the lungs to pick up oxygen. This can lead to failure of the right side of the heart.
Interstitial lung disease (ILD), also called pulmonary fibrosis, is another serious complication.
An estimated 80,000 to 100,000 in the U.S. have the systemic form of scleroderma with many more people diagnosed with the localized form of the disease.
Like many autoimmune disorders, the exact cause or causes of scleroderma are unknown.
Skin hardening is one of the most visible manifestations of the disease. Other symptoms can range from mild (heart-burn) to life-threatening (shortness of breath). The severity depends on which parts of the body are affected, and to what extent.
When the lungs are involved, those with mild pulmonary hypertension (PH) may have no symptoms. People with moderate or severe PH usually notice shortness of breath, especially with exercise. You may also notice unusual chest pains and symptoms of right-sided heart failure, such as worsening shortness of breath and swelling of the feet and legs. Other symptoms may include cough, light-headedness or fainting, palpitations (heart racing or fluttering) and swelling.
As with many autoimmune disorders, people with Scleroderma may have other autoimmune diseases as they often present in “clusters.” About 90% of people with systemic scleroderma also have Raynaud Phenomenon, the constriction of blood vessels in the ears, nose, fingers or toes. This can cause the affected areas to turn red and tingle or become numb.
Diagnosis and Treatment
The diagnostic process may require a visit with a rheumatologist or a dermatologist and a blood study or other specialized tests depending on which organs are affected.
Currently, there is no cure for scleroderma, but there are treatments available. Some are directed at symptoms while others are aimed at depressing the immune system.
Some people with a mild form of the disease may not need any medication, and occasionally, people can go off treatment when their scleroderma is not active.
Because there is so much variation from one person to the next, treatments vary for each patient.
Lung disease is a major cause of scleroderma-related deaths. Research indicates that 25 percent of scleroderma patients will die from pulmonary hypertension; another 23 percent will die from pulmonary fibrosis.