Diffuse Systemic Scleroderma

Diffuse systemic scleroderma is a chronic autoimmune condition that is a subtype of systemic scleroderma (the other being limited scleroderma). 

Diffuse vs. limited systemic scleroderma

Patients with diffuse systemic scleroderma are more likely to have extensive skin fibrosis on the arms, legs, and trunk and a higher risk of developing internal organ damage, compared to those with limited scleroderma. Those with diffuse systemic scleroderma also experience symptoms that worsen quickly and are more severe. About 35 percent of patients with systemic scleroderma develop the diffuse form of the disease.

Symptoms of diffuse systemic scleroderma

The tightening of the skin may make it difficult for patients to bend their fingers, a condition known as sclerodactyly, as well as their hands and other joints. The skin can lose or gain pigment, become swollen, and be itchy. Patients also may experience their fingers turning white, blue, and cold when they feel stressed or are in cold environments, a condition known as Raynaud’s phenomenon.

Sometimes joints, tendons, and muscles become inflamed and painful. People with diffuse systemic scleroderma often feel tired, have a diminished appetite, and experience weight loss. The heart and kidneys   also may be damaged in those with diffuse systemic scleroderma.

Scar tissue may build up in the lung (lung fibrosis) and cause interstitial lung disease (ILD). Pressures in the arteries supplying blood to the lung may rise to very high levels, causing another lung condition known as pulmonary arterial hypertension (PAH). These conditions are the main causes of life-threatening scleroderma.

Symptoms of diffuse systemic scleroderma can vary greatly among patients. Sometimes people with the disease enter a phase where disease progression stabilizes. This phase usually begins three to five years after diagnosis and can last for different lengths of time.

Diagnosing diffuse systemic scleroderma

Differentiating between diffuse and limited systemic scleroderma is done mainly by measuring the extent of skin symptoms. Physicians may order blood tests for specific autoantibodies (those working against the body’s own tissues and organs,) CT scans of the lungs, tests to assess breathing capabilities, urine tests to check the function of the kidneys, or an echocardiogram to assess the heart.

Treatment and prognosis of diffuse systemic scleroderma

There is no cure, so far, for diffuse systemic scleroderma, but treatments are available to ease symptoms, to prevent the worsening of the disease, and to improve patients’ overall quality of life. Treatment regimens may differ between individuals depending on the organs that are affected. Although the disease may lie dormant for several years, there is no certainty whether symptoms will improve, remain the same, or worsen with time.


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