Scleroderma

The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) says scleroderma (or schleroderma) is both a rheumatic disease that can cause inflammation and pain in the muscles and joints (Read about "Arthritis and Rheumatic Diseases"), as well as a connective tissue disease that can cause thickening, hardening or tightening of the skin, blood vessels and internal organs. (Read about "Skin" "Vascular System") The Scleroderma Foundation says scleroderma affects about 300,000 people in the U.S., and that women are four times more likely to develop it than men.

Scleroderma is a chronic disease, which occurs because the body produces too much of a protein called collagen. Excess collagen is deposited causing thickening and hardening. There are two main classes of scleroderma:

• Localized scleroderma - In localized scleroderma, the skin develops either hard, oval patches (morphea) or lines or streaks (linear). According to the Scleroderma Foundation, this type is more common in children. Often, localized conditions improve or go away on their own over time, but the skin changes and damage that occur when the disease is active can be permanent. For some people, localized scleroderma is serious and disabling.
• Systemic scleroderma - In generalized or systemic scleroderma, also called systemic sclerosis, the thickening and hardening affect connective tissue throughout the body, including the skin, the esophagus, gastrointestinal tract, lungs, kidneys, heart and other internal organs. It may also affect blood vessels, muscles and joints.

NIAMS says one of the most common problems associated with systemic scleroderma is Raynaud's phenomenon (Read about "Raynaud's Phenomenon"), creating the potential for painful skin ulcers which can be made worse by smoking. (Read about "Quit Smoking") In addition, some people with systemic scleroderma develop severe lung disease, including pulmonary fibrosis (hardening or scarring of lung tissue because of excess collagen) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs). (Read about "Pulmonary Hypertension") Others with systemic sclerosis may develop heart problems; including scarring and weakening of the heart and abnormal heart beat (Read about "Cardiomyopathy" "Arrhythmias"). Kidney problems can also occur. (Read about "Kidney Disease") Scleroderma can also produce abnormal sensitivity to cold, as well as oral and dental problems (Read about "Oral Health"), fatigue and/or weight loss. It's essential that people with scleroderma discuss potential problems with their doctor.

Currently, there is no treatment that controls or stops the underlying problem - the overproduction of collagen - in all forms of scleroderma. Thus, treatment and management focus on relieving symptoms and limiting damage. Your treatment will depend on the particular problems you are having.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors involved in your care. Typically, care will be managed by a rheumatologist (a doctor specializing in treatment of musculoskeletal disorders and rheumatic diseases). Your rheumatologist may refer you to other specialists, depending on the specific problems you are having. For example, you may see a dermatologist for the treatment of skin symptoms. Dentists, orthodontists and even speech therapists can treat oral complications that arise from thickening of tissues in and around the mouth and on the face. People with systemic scleroderma may need to see a nephrologist for kidney complications, a cardiologist for heart complications, a gastroenterologist for problems of the digestive tract or a pulmonary specialist for lung involvement.

NIAMS says self-help measures include dressing warmly, and not smoking or quitting if you do smoke. "Quit Smoking" Gentle stretching exercises or special classes may be helpful. Good oral hygiene is important, as is good skin care. (Read about "Oral Health" "Skin Care") A rehabilitation specialist can provide information on physical therapies that may be helpful. (Read about "Rehabilitation")