What is the role of imaging studies in the workup of pediatric mixed connective-tissue disorder (MCTD)?

Updated: Oct 24, 2018
  • Author: Marisa S Klein-Gitelman, MD, MPH; Chief Editor: Lawrence K Jung, MD  more...
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Answer

See the list below:

  • Initial imaging studies should include the following:

    • Chest radiography should be performed.

    • Barium swallow should be performed to evaluate esophageal motility.

    • Echocardiography should be performed to evaluate myocardial and valvular function and to obtain an estimate of pulmonary artery pressure. Obtain baseline echocardiogram to look for evidence of myocarditis, valvulitis, and pulmonary hypertension. Of particular importance, monitor for pulmonary hypertension over time. Early diagnosis may lead to better response to treatments with corticosteroids. Currently, the use of sildenafil is under investigation to determine therapeutic benefit.

  • In addition to chest radiography, pulmonary function tests, and clinical symptoms, high-resolution CT scan of the lung may be necessary to determine if pulmonary fibrosis exists. The presence of interstitial lung disease is often missed. [13] Screening for this problem is important as it is more readily treated early in the disease course.

  • Other imaging studies should be guided by clinical manifestations and may include the following:

    • Brain MRI

    • Renal ultrasonography, nuclear medicine evaluation of renal function, or both

    • Plain films to evaluate arthritis

  • Inhaled aerosol clearance times of Tc 99m–labeled diethylene-triamine penta-acetate (Tc 99m–DTPA) should be obtained to evaluate pulmonary interstitial fibrosis and improvement after treatment.


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