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Scleroderma

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.

Alternative Names

Systemic Sclerosis

Diagnosis

The physician may suspect scleroderma after taking a history of the symptoms and performing a physical examination. As part of this examination, the physician does the following:

  • Checks the skin for thickened and hardened areas. The major clinical clues to scleroderma are symmetrical hardening and thickening of the skin in any areas on the fingers and toes.
  • Presses affected tendons and joints to detect crackling or grating sensations, which can indicate sclerodermic changes beneath the skin.
  • Examines the fingernails underneath a microscope. The physician may detect changes in capillaries that are characteristic of scleroderma and mixed connective tissue disease.

Tests for Antinuclear Antibodies

Tests may be conducted to detect immune factors called antinuclear antibodies (ANAs). Elevated levels of ANA are found in 95% of patients with scleroderma. Antinuclear antibodies, however, are also strongly present in patients with many other autoimmune diseases, including systemic lupus erythematosus. Many people can also have high levels and never develop any of these diseases.

Detecting ANA subtypes may provide some weight in diagnosing scleroderma. Such subtypes include the following:

  • Anti-topoisomerase antibodies.
  • Anti-centromere antibodies. The centromere is an important factor in cell division, and antibodies to centromeres are found in between 50% and 80% of people with CREST.

These antibodies are also found in other rheumatologic disorders, however; so detecting them does not necessarily prove scleroderma.

Ultrasound

High frequency ultrasonography may be used to detect effects of scleroderma in patients hands.

Diagnosing Systemic Complications

Diagnosing Lung Complications. Changes in the lungs may occur early in lung disease, and prompt treatment is critical to prevent complications. Therefore once a diagnosis is made, the physician will check for lung changes:

  • Using a stethoscope the physician will first listen for specific lung sounds. Rales (a crackling sound) heard at the base of the lungs while inhaling is an indicator of pulmonary fibrosis even if breath function is normal.
  • Respiratory function tests determine lung capacity.
  • A chest x-ray is usually performed. X-rays, however, do not always detect lung disease, particularly in children.
  • Patients also are often asked to inhale nitric oxide to test the ability of blood vessels to open. (According to a small 2002 study, this test may cause complications in patients with CREST syndrome, who should be tested with caution.)
  • More extensive tests, notably high resolution computed tomography (CT) scans and bronchoalveolar lavage, may be needed if severe lung scarring is suspected.
  • Echocardiography is a noninvasive and very helpful imaging technique for detecting pulmonary hypertension, an uncommon but life-threatening complication of severe scleroderma. However, only an invasive procedure called right-heart catheterization will confirm the diagnosis of this condition.
Swan Ganz catheterization
Right heart catheterization involves the passage of a catheter (a thin flexible tube) into the right side of the heart to obtain diagnostic information about the heart and for continuous monitoring of heart function in critically ill patients.

Diagnosing Heart Complications. Patients with suspected heart involvement should have electrocardiography, echocardiography, or radionucleotide ventriculography. Advanced imaging techniques, such as single photon emission tomography (SPET), may also be useful to determine the extent of heart involvement in scleroderma patients.

Diagnosing Gastrointestinal Complications. Gastrointestinal problems may be detected using endoscopy (the insertion of a tube down the esophagus that contains a small camera and other instruments) or with manometry, a test that measures the pressure exerted by the muscles in the esophagus. Electrogastrography (EGG) measures the electrical activity in muscle in the stomach and may be an effective method for detecting stomach problems.

Gastric endoscopy Click the icon to see an image about endoscopy.

Ruling out Other Conditions

Other Autoimmune and Connective Tissue Disorders. A number of other autoimmune conditions affect connective tissue and can strongly resemble and even occur at the same with scleroderma. They include the following:

  • Rheumatoid arthritis.
  • Systemic lupus erythematosus.
  • Polymyositis.

Symptoms of such diseases also may include fever, arthritis, muscle aches, rash, and lung and heart involvement.

Eosinophil Fasciitis. Eosinophilic fasciitis is a muscle disorder that is known to occur after intense exertion. It can cause symptoms similar to scleroderma, including pain, swelling, and tenderness in the hands and feet as well as skin thickening. The disorder can be ruled out if blood tests show elevated sedimentation rate and no antinuclear antibodies.

Conditions Associated with Raynauds phenomenon and other Symptoms of Scleroderma. Although Raynauds phenomenon occurs in most scleroderma patients, over 80% of cases of Raynauds phenomenon are harmless. In one study, only 12% of Raynaud's cases was associated with some other condition, and few of those are scleroderma. The following are other problems that might accompany or cause Raynaud's phenomenon:

  • Other autoimmune connective tissue diseases.
  • Diabetes. Patients with diabetes may develop Raynauds phenomenon and other scleroderma-like symptoms.
  • Certain drugs, including bleomycin, ergot derivatives (used for migraines), and methysergide.
  • Hereditary hemorrhagic telangiectasia. This is very similar to the CREST syndrome and poses a diagnostic dilemma for the physician, although it is very rare.
  • Keloids are areas of scarring overgrowth on the skin that develop at the site of skin injuries. They are caused by genetic factors and are most likely to occur in African Americans. Extensive keloids may be mistaken for scleroderma.
Keloid Click the icon to see an image of a keloid.
  • Repetitive stress injuries (particularly from vibrating tools).
  • Hypothyroidism.
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