Scleroderma is a rare disease of the autoimmune system that many people haven’t heard of. Yet recognizing the symptoms can help you get treatment faster – and provide the best chance for a successful outcome.
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The disease is characterized by hard skin — “sclero” means “thickening” and “derma” means “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.
Localized scleroderma affects only the skin. Systemic scleroderma, also called sclerosis, affects the skin and internal organs, including the digestive system, lungs, kidneys, heart, blood vessels, muscles and joints.
Systemic scleroderma falls into two major categories. One is limited cutaneous scleroderma, which usually affects the skin below the elbow and knee but can include the neck and face.
The second type is diffuse cutaneous scleroderma, which affects the skin above the elbows and knees and includes the trunk. Both types of scleroderma can affect internal organs such as the lungs, heart, kidneys and esophagus.
Although scleroderma often is mild, in its most severe forms it can be life-threatening. Its signs and symptoms vary, depending on which part of the body the disease affects.
Awareness of the disease and its symptoms is important because its symptoms are similar to other autoimmune diseases, says pulmonologist Kristin Highland, MD. Dr. Highland specializes in treating scleroderma-associated lung disease.
“Two of the more serious complications of scleroderma are interstitial lung disease, or pulmonary fibrosis, and pulmonary hypertension” Dr. Highland says. “Quick and proper diagnosis and treatment can help to minimize these complications and decrease the chance of irreversible damage.”
With June being Scleroderma Awareness Month, here are four commonly asked questions – and answers – about scleroderma:
How common is scleroderma?
In the United States, scleroderma affects about 240 of every million people. Every year, 19 people out of a million are diagnosed with scleroderma.
The disease is more common in women. Up to one in four people with scleroderma are women.
The disease most often strikes before age 40. But anyone can develop scleroderma – even children, Dr. Highland says.
Interstitial lung disease and pulmonary hypertension are the leading cause of illness and death in scleroderma.
What causes scleroderma?
The cause of scleroderma is unknown. Most patients do not have any relatives with scleroderma and their children do not get scleroderma.
Research indicates there is a susceptibility gene, which raises the likelihood of getting scleroderma, but the gene by itself does not cause the disease, Dr. Highland says.
Scleroderma is not contagious.
What are the symptoms of scleroderma?
- Raynaud phenomenon – often the first symptom
- Swollen hands and feet
- Thickened skin
- Red spots on the skin
- Calcium deposits in the skin
- Stiff joints
- Tight, mask-like facial skin
- Painful sores on the fingertips and toes
- Pain and stiffness in the joints
- A cough that won’t go away
- Shortness of breath
- Difficulty swallowing
- Digestive problems
- Weight loss
- Hair loss
How is scleroderma treated?
Although no cure has been found for scleroderma, the disease very often progresses slowly and is manageable. Many people who have it may lead healthy and productive lives. Treatments are available for many of the disease’s symptoms and complications.
“Scleroderma can have many symptoms, and so a combination of approaches often is necessary to treat and manage the disease effectively,” Dr. Highland says.
For instance, scleroderma can result in heartburn or affect the bowel. Medicine can control both of these symptoms.
There are many medications to treat pulmonary hypertension, and physicians often prescribe immunosuppressive therapy for interstitial lung disease. Some people with mild disease may not need medication at all and occasionally people can go off treatment when their scleroderma is no longer active.
Like many other conditions, education about scleroderma and local support groups can be the greatest tools for managing the disease and reducing the risk of further complications.