Understanding Scleroderma

What is SCLERODERMA?

Scleroderma is an autoimmune, rheumatic, and chronic disease that affects the body by hardening connective tissue.  (“Connective tissue” is widespread.  It adds strength to organs and other parts of the body.)  Connective tissue is made of many kinds of proteins.  Among them is collagen of the skin.  Scleroderma literally means hard skin.

An Illness of the Immune System

Scleroderma is classified as an autoimmune disease. This means that a person’s immune system works against itself. The normal immune system protects the body by fighting off foreign invaders such as viruses and infections. In an autoimmune disease, the immune system mistakes a person’s own tissues as foreign invaders and sets up a protective attack that backfires to cause problems.

In scleroderma, cells start making collagen as if there were an injury that needs repairing. The cells do not turn off as they should and end up making too much collagen. The extra collagen in the tissues can prevent the bodies organs from functioning normally.

An Illness that Varies from Person to Person

The severity of scleroderma varies from person to person.   It can be a mild annoyance, or it can cause significant clinical problems.  For others, it can become life threatening. Most people have episodes where the illness improves or even goes into remission.

An Illness That Does Not Go Away

Scleroderma is chronic. This means that it lasts for your lifetime. However, like diabetes, high blood pressure, heart disease, and psoriasis, scleroderma can be treated and the symptoms managed.  The cause of scleroderma is unknown and there is currently no cure, but there are treatments that can effectively prevent or limit the damage caused by this chronic disease.

Who gets SCLERODERMA?

Only a few people get scleroderma, but no one really knows why.
Scleroderma is a rare disease. Fewer than 500,000 people in the United States have scleroderma. No one knows for sure why or how it develops. Some experts report that six out of seven patients are women.  The most common age span for scleroderma to develop is between 35 and 50. Still, young children and older adults can get the disease.

Some families are more affected by autoimmune disorders than others.  Although scleroderma does not seem to run in families, it is common for family members to have other autoimmune diseases like thyroid disease, rheumatoid arthritis, or lupus. African Americans and Native Americans generally have more severe scleroderma than Caucasians. These findings suggest a hereditary (genetic) component to scleroderma and other autoimmune disorders.

Environmental factors could also put people at risk for scleroderma. For example, men exposed to silica appear to have a higher risk for developing scleroderma and certain drugs are capable of causing a scleroderma-like reaction.

EARLY SYMPTOMS

Changes in fingers.

For some people, two symptoms are among the early signs of scleroderma:

  • Fingers that become very sensitive to cold and change color with cold or emotional stress: Raynaud’s phenomenon
  • Fingers and hands that become stiff and puffy

Finger color changes are caused by spasm and narrowing of blood vessels. This occurs because of excess collagen that has narrowed the blood vessels and over reaction of the skin blood vessel to cold temperatures and emotional stress.

The cold sensitivity and color changes are called Raynaud’s (pronounced RAY-KNOWDS) phenomenon. Raynaud’s phenomenon is a common condition. Most people with Raynaud’s phenomenon will NOT develop scleroderma. There are two types of Raynaud’s phenomenon:

1. Primary Raynaud’s

People who have Raynaud’s phenomenon and not scleroderma or other autoimmune disorders have primary Raynaud’s.

2. Secondary Raynaud’s

A small percentage of people with Raynaud’s phenomenon develop scleroderma. Conversely, almost everyone with scleroderma has Raynaud’s symptoms. This is secondary Raynaud’s phenomenon, so-named because it is secondary to the scleroderma.

Is All Scleroderma the Same?

No. Not everyone has the same experience.

How scleroderma progresses and changes over time varies greatly among people.  Most people experience tight and swollen fingers in the beginning and will likely have Raynaud’s phenomenon. Afterwards, it can take months to years for the full extent of the disease to develop. In many patients, it remains being mild and does not progress.  In general, the more skin that is involved, the more severe the case of scleroderma.

Learn more about the different types of Scleroderma.