What Is Scleroderma?

Scleroderma is a chronic autoimmune disease that was first described in the 18th century. The term scleroderma means "hard skin," which describes thickening of the skin from increased deposits of collagen.

There are two types of scleroderma. Localized scleroderma affects the skin in limited areas and the musculoskeletal system. Systemic sclerosis causes more widespread skin changes and may be associated with internal organ damage in the lungs, heart and kidneys. It can cause arthritis, slow contractions in the gastrointestinal tract, muscle inflammation, dry eyes and dry mouth. Most people with scleroderma have cold-induced spasms of small blood vessels in their hands or feet, known as Raynaud’s phenomenon, which caused the fingers or toes to turn white or blue and may be painful.

Cause

In most cases, the cause of scleroderma is unknown. However, in a small minority of cases, scleroderma or scleroderma-like illnesses are associated with exposure to certain toxins or as a complication of bone marrow transplants. Scleroderma is not contagious and is rarely inherited.

Systemic sclerosis is associated with over-activation of the immune system, which normally functions to protect the body against cancers and invading infections. This causes damage to cells that line small blood vessels, which in turn leads to the over-production of scar tissue.

Health Impact

- Scleroderma affects women more than men and adults more than children.
- 10-20 new cases are diagnosed per million people each year.
- Five-year survival rate is 80 – 85 percent.
- Lung, heart and kidney damage are the most frequent causes of severe disability and death.
- Many people have decreased hand function because of joint disfigurement or finger ulcers.
- Diagnosis

Diagnosis of scleroderma is based on clinical history and physical findings. Diagnosis may be delayed in those without significant skin thickening. Laboratory, X-ray and pulmonary function tests determine the extent and severity of internal organ involvement.

Treatment

There is no known cure for scleroderma. No treatment has been scientifically proven to alter the overall course of the disease, although d-penicillamine is commonly used for this purpose and may be of some value.

There are a number of effective organ-specific treatments for scleroderma. Raynaud’s phenomenon may be helped by calcium channel blockers. Declining renal function and hypertension are often treated with drugs. Esophageal damage from reflux of stomach contents can be treated with acid-reducing drugs. Antibiotics, special diets and medication can improve absorption of nutrients in people who have abnormalities of their intestines. Musculoskeletal pain may respond to nonsteroidal anti-inflammatory agents. Lung inflammation may be treated with cyclophosphamide.

Physical and occupational therapies are used to minimize joint disability and functional impairment.

The Rheumatologist’s Role In Treating Scleroderma

Rheumatologists are specially trained in caring for people with autoimmune diseases such as scleroderma. Because many different organs may be involved, the evaluation and treatment of scleroderma is best managed by a rheumatologist. Rheumatologists in basic and clinical research are the leaders in improving our understanding and treatment of this rheumatic disease.