Friday, February, 10, 2012

Scleroderma - Prognosis

Table of Contents

  • Anti-U1-RNP and anti U3-RNP are associated with muscle inflammation.
  • ACA is commonly associated with pulmonary hypertension and vascular disease.
  • TOPO is associated with pulmonary fibrosis.
  • RNA Polymerase III (Pol 3) is rarely linked to severe interstitial fibrosis, although this autoantibody is strongly present in patients with kidney crisis.
  • Patients with diffuse scleroderma who have Pol 3 have the best survival rates.

Diagnosing Systemic Complications

Diagnosing Lung Complications. Changes in the lungs may occur early in scleroderma lung disease, and prompt treatment is very important to prevent complications. For this reason, once a diagnosis is made, the doctor will check for lung changes in the following ways:

  • Listen to the lungs through a stethoscope. Rales, a crackling sound at the base of the lungs as the patient breathes in, is a sign of pulmonary fibrosis, even if breath function is normal.
  • Perform respiratory function tests to determine lung capacity.
  • Take a chest x-ray (however, x-rays do not always find lung disease, especially in children).
  • Have patients inhale nitric oxide to test the ability of blood vessels to open.
  • Perform more extensive tests, such as high-resolution computed tomography (CT) scans and bronchoalveolar lavage, if there is a suspicion of severe lung scarring.

Newer tests showing promise in diagnosing lung complications include:

  • The induced sputum test, which looks at cells taken from coughed-up phlegm
  • A test that uses an inhaled chemical, technetium-labeled diethylenetriamine pentaacetate (99mTC-DTPA), to detect lung damage

Diagnosing Heart Complications. Patients with suspected heart complications should have the following tests:

  • Electrocardiography (ECG): A test of the heart's electrical activity
  • Echocardiography: A look at the beating heart through the use of sound waves
  • Radionucleotide ventriculography: An evaluation of the working heart using a radioactive dye

Review Date: 01/04/2011
Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)