Scleroderma is a chronic, progressive rheumatic disease that causes abnormal growth of connective tissue in the body. Chronic means that it persists over a long time. Progressive means that it gets worse over time.
Scleroderma is also considered an autoimmune disease, which means it’s caused by the body attacking its own healthy tissues. As a result, the body produces too much collagen, the protein that forms the basis of connective tissue. This causes a thickening and scarring of these tissues. It can affect the joints, skin and internal organs.
There are two main types of scleroderma:
- Localized scleroderma occurs in one area of the body on the skin, and some symptoms may improve over time. It doesn’t affect the internal organs.
- Systemic scleroderma affects the entire body; over time, symptoms can damage the skin and cause it to thicken and tighten, changing your appearance and restricting movement. It can also cause severe kidney, lung, digestive or heart problems, which can be fatal.
Although scleroderma isn’t directly inherited, some scientists feel there’s a slight predisposition to it in families with a history of rheumatic diseases, suggesting a genetic component. Many patients with scleroderma don’t have family members with the disease, however, suggesting that environmental factors also play a role.
Scleroderma is considered a rare disease, with fewer than 500,000 Americans estimated to have it. Most of them are women between 35 and 50.
Because scleroderma is such a complex disease and its symptoms can look like other conditions, it’s important to work with specialists who have advanced training and experience in diagnosing and treating it—which is exactly what you’ll find at Cooper.
Why Choose Cooper to Diagnose and Treat Scleroderma
Cooper University Health Care’s Division of Rheumatology has a team of expert, board-certified rheumatologists with extensive experience in diagnosing and treating scleroderma. Our capabilities include:
- Thorough diagnostic testing: Diagnosis is based on changes in the skin and internal organs, and begins with a comprehensive medical history and physical exam. A blood test called an antibody test may help determine the specific type of scleroderma you have. Other tests may include:
- Electrocardiogram (EKG or ECG) to detect changes in heart muscle tissue
- Echocardiogram to view the heart’s structure and function
- X-ray to show changes in bone, soft tissues and organs caused by scleroderma
- Pulmonary function test to evaluate lung function
- A multidisciplinary team approach to care: Because scleroderma can affect so many different body systems, you may need a variety of specialists on your care team. As an academic health system, Cooper has physician experts in more than 75 specialties, giving you convenient access to all the expertise you need, all in one place.
- Personalized treatment based on your symptoms, age and general health, as well as the type of scleroderma you have and how severe the condition is. Treatments for scleroderma include:
- Medications such as pain relievers, anti-inflammatories, and immunosuppressive drugs to slow the skin-thickening process and delay damage to internal organs
- Management of specific symptoms such as Raynaud’s phenomenon , respiratory issues or heartburn
- Physical therapy and exercise to maintain muscle strength
- Psychological counseling to provide emotional support
Scleroderma Causes and Risk Factors
No one knows exactly why or how scleroderma develops, but medical researchers are working hard to understand its cause. What we do know:
- The majority of patients are women
- Scleroderma most commonly develops between the ages of 35 and 50, but children and older adults can get the disease
- Scleroderma doesn’t seem to run in families, but it is common for family members to have other autoimmune diseases such as rheumatoid arthritis, lupus, or thyroid disease
- African Americans or Native Americans generally have more severe scleroderma than Caucasians
- Men exposed to silica dust (often in agricultural or industrial settings) appear to be at higher risk for developing scleroderma
- Certain medications (including some chemotherapy drugs) may cause a scleroderma-like reaction in some patients
As collagen overproduction causes scarring and thickening of connective tissue, it produces symptoms that can vary from person to person. These symptoms may include:
- Skin changes
- Skin hardening and thickening, particularly on fingers, toes, hands, arms and face
- Hair loss in affected areas
- Skin color changes
- Sores/ulcers on fingertips and knuckles
- Swelling in hands or feet
- Calcium bumps (calcinosis) on fingers or other bony areas
- Immovable fingers, wrists or elbows due to skin thickening
- Tight, shiny, darker skin on large areas that may restrict movement
- Raynaud’s phenomenon, when blood flow to fingers, toes, tips of ears and other areas is reduced due to blood vessel spasms in response to cold, stress or emotional upset
- Heart and lung problems
- Abnormal heart rhythms
- Shortness of breath
- Chest pain
- Persistent cough
- Pulmonary hypertension (high blood pressure in the arteries to the lungs)
- Arthritis and muscle weakness
- Joint pain, stiffness and swelling
- Kidney issues
- Kidney failure
- High blood pressure
- Digestive problems
- Gastroesophageal reflux disesase (GERD), often called heartburn
- Difficulty swallowing due to scarring of the esophagus
- Nausea or vomiting
- Weight loss
- Spider veins (telangiectasia)
- Tiny blood vessels become dilated and show through the skin as small reddish spots on fingers, palms, face, tongue and lips
Because scleroderma symptoms can look like other health disorders, it’s important to see a rheumatology specialist for an accurate diagnosis.
While there’s no cure to stop the collagen overproduction this autoimmune disease causes, there are medications that can help manage symptoms and help prevent complications:
- Pain relievers (both over-the-counter and prescription) can help you manage discomfort
- Blood pressure medication may help dilate blood vessels, helping prevent kidney and lung problems, and treat Raynaud’s phenomenon
- Immunosuppressive drugs may help reduce scleroderma symptoms and slow disease progression
- Acid reflux medications can help relieve digestive symptoms
- Antibiotic ointment can help prevent infection of fingertip sores caused by Raynaud’s phenomenon
- Influenza and pneumonia immunizations can help protect lungs damaged by scleroderma
- Appropriate exercise can help you manage pain, reduce stiffness, improve strength and mobility, and maintain your independence to perform daily tasks
- Avoid tobacco, alcohol, caffeine and recreational drugs
- Get enough sleep
- Minimize stress and manage anxiety
- Avoid processed and high-sugar foods and drinks
- Dress warmly in cold weather to minimize circulation problems
- Eat smaller meals to minimize the effects of GERD