Learning About Scleroderma
June is Scleroderma Awareness Month. A serious autoimmune rheumatic disease, scleroderma affects about 300,000 Americans, and it has a mortality rate of 20-50%.1
Who it affects
Scleroderma can develop in every age group from infants to the elderly, but most patients start experiencing symptoms between the ages of 25 to 55. And it’s more common in women, with female patients with scleroderma outnumbering males by about 4-to-1. Like many other autoimmune diseases, symptoms and severity vary from patient-to-patient and diagnosis isn’t easy—all the more reason to shed light on scleroderma and how it manifests.2
What it is
Scleroderma is a chronic disease that involves the hardening and tightening of the skin and connective tissues. It results from an overproduction and accumulation of collagen in body tissues. There are two types of scleroderma:
- Localized scleroderma. This most common type is relatively mild and affects only skin tissues. For some, it gets better or goes away without treatment; others may experience more severe symptoms and skin damage.
- Systemic scleroderma. This type affects about one-third of patients. It results in the hardening of their skin, the tissues under their skin, blood vessels, muscles, joints, and major organs including the stomach and bowels, lungs, kidneys and heart.2
How it feels
Most people with this disease feel hardening and tightening of the skin that might restrict their movement. This symptom is also the most visible, too, presenting with shiny skin patches shaped like ovals, straight lines, or covering wide area of their trunks or limbs.
Some people feel an exaggerated response—pain, numbness or color changes—in their fingers or toes when they’re experiencing cold temperatures or emotional distress. If their digestive system is affected, people may have acid reflux; if their intestinal muscles aren't moving food properly through the intestines, they’ll have trouble absorbing nutrients. When the disease affects a person’s heart, lung or kidneys function, they can suffer very serious symptoms to varying degrees. 3
How it’s diagnosed and treated
Rare autoimmune diseases can be a challenge to diagnose. Doctors take into account a patient’s medical history, a physical exam, lab tests and a skin biopsy. A case that’s diagnosed and treated early has a better chance of not becoming more serious, but scleroderma has no cure—yet. There is no drug on the market that can stop the overproduction of collagen, but there are various medications available to help patients control their symptoms and prevent complications. These include drugs that dilate blood vessels, suppress the immune system, reduce stomach acid and relieve pain.
In January 2018, the Hospital for Special Surgery (HSS) released promising study results that may point to the cause of scleroderma and may even provide leads for a treatment. 4
A lifestyle that emphasizes overall health and wellness, including eating a nutritious diet, not smoking, exercising and taking good care of the skin, can help patients feel their best and maintain independence with daily tasks.
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References:
1 “New HSS Study May Signal Hope for Patients Suffering from Scleroderma.” News-Medical.net, News-Medical.net, 11 Jan. 2018, news-medical.net/news/20180111/New-HSS-study-may-signal-hope-for-patients-suffering-from-scleroderma.aspx.link opens in a new window
2 “What Is Scleroderma?” What Is Scleroderma? - Scleroderma Foundation, scleroderma.org/site/PageNavigator/patients_whatis.html#.WwWJ9O7RW1s.link opens in a new window
3 “Scleroderma.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 21 June 2016, mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952.link opens in a new window
4 “New HSS Study Finds Hope in Understanding and Better Treating Scleroderma.” Hospital for Special Surgery, hss.edu/newsroom_scleroderma-hss-research.asp.link opens in a new window