Scleroderma

What is scleroderma?

Scleroderma is an autoimmune disorder in which the skin and connective tissues tighten and harden. There are several different types of scleroderma. It is a long term disease that worsens over time.  
 
Scleroderma occurs when the immune system becomes overactive and produces too much collagen – the protein that forms the basis of connective tissue. This results in a thickening (fibrosis) and scarring of tissue. Scleroderma is not contagious. It may run in families, but often occurs in patients without any family history of the disease. It ranges from very mild to potentially fatal.
 
The two main types of scleroderma are: 
 
  • Localized scleroderma usually affects only the skin, although it can spread to the muscles, joints, and bones. Internal organs are not affected. Symptoms include discolored patches on the skin (morphea); or streaks of thick, hard skin on the arms and legs (linear scleroderma). 
  • Sytemic scleroderma is the most serious form of the disease and can affect the skin, muscles, joints, blood vessels, lungs, kidneys, heart, and other organs. There are two main forms of systemic scleroderma: limited cutaneous systemic sclerosis (CREST syndrome) and diffuse cutaneous systemic scleroderma.
 
 
Risk factors
 
  • Age: occurs most often between ages 35-50 
  • Sex: 6 out of 7 patients are women
  • Genetics: scleroderma does not seem to run in families, but it is common for family members to have other autoimmune disease like thyroid disease, rheumatoid arthritis, or lupus
  • Race/ethnicity: African Americans and Native Americans generally have more severe scleroderma than Caucasians.
  • Environmental factors such as exposure to silica in men and certain drugs can cause a scleroderma-like reaction
 
 
Diagnosis 
Diagnosis of scleroderma can be difficult because symptoms may be similar to those of other diseases. A doctor will ask about the patient’s medical history, perform a physical exam, and might order lab tests and X-rays. Careful clinical evaluation is the primary method for monitoring scleroderma. Tests that may be ordered by your doctor include:
 
  • Computerized tomography (CT) scans are used to look at bone abnormalities. 
  • Thermography scans can detect differences in skin temperature between the lesion and normal tissue. 
  • Ultrasound and magnetic resonance imaging (MRI) can aid soft tissue assessment
 
Symptoms your doctor will for include:
 
  • Raynaud’s phenomenon: color changes (blue, white, and red) in the fingers and toes – often after exposure to cold. One of the earliest signs of the disease.
  • Skin thickening, tightening, and swelling: This is symptoms that gives the condition its name (“sclera” means hard, and “derma” means skin). Skin may also become glossy or unusually dark or light in places.
  • Enlarged red blood vessels on the hands, face, and around nail beds (called “telangiectasias”)
  • Calcium deposits in the skin or other areas.
  • High blood pressure from kidney problems
  • Heartburn
  • Other problems of the digestive tract such as difficulty swallowing, bloating, and constipation
  • Shortness of breath
  • Joint pain
 
 
Treatment
There is no cure for scleroderma, but treatments are available to help manage symptoms, including medications and lifestyle modifications. Symptoms can vary greatly from one person to another, so treatment options are very broad. A rheumatologist will likely be the leading doctor of the healthcare team. Other specialists may also be needed to treat heart, kidney, skin, lung, dental, and gastrointestinal problems that occur. 
 
 
 
# SCLERODERMA BY THE NUMBERS #
  • Scleroderma is relatively rare, affecting about 75,000 to 100,000 people in the US
  • Approximately 50,000 Americans are affected by systemic sclerosis 
  • Systemic sclerosis is 2-3x more common in women than men
  • Raynaud’s phenomenon affects nearly 90% of people with scleroderma at some point in their life
 
about scleroderma
 
 

Sources:
  • “Scleroderma.” Rheumatology.org, www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma.
  • “Understanding Scleroderma • Johns Hopkins Scleroderma Center.” Johns Hopkins Scleroderma Center, www.hopkinsscleroderma.org/scleroderma/.
  • “Scleroderma.” Www.arthritis.org, www.arthritis.org/about-arthritis/types/scleroderma/.
  • “Scleroderma.” Mount Sinai Health System, www.mountsinai.org/health-library/diseases-conditions/scleroderma.
  • “NIH Fact Sheets - Scleroderma.” National Institutes of Health, U.S. Department of Health and Human Services, report.nih.gov/NIHfactsheets/ViewFactSheet.aspx?csid=68.

 

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