What is included in the long-term monitoring of mixed connective-tissue disorder (MCTD)?

Updated: Oct 01, 2018
  • Author: Eric L Greidinger, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Patients with stable disease and no recent changes in medications should be seen approximately every 2-4 months and undergo routine laboratory evaluation, including a complete blood cell count and chemistry studies. Patients with active disease are typically seen approximately every 3-6 weeks, depending on the severity of disease.

Recommendations for screening and early detection of pulmonary artery hypertension (PAH) associated with connective tissue diseases, including MCTD, have been published. [26] Although these guidelines do not recommend screening of asymptomatic patients with MCTD who lack features of scleroderma, the following studies are recommended for symptomatic patients:

  • Pulmonary function testing (spirometry with lung volumes) with single-breath diffusing capacity for carbon monoxide (DLCO)
  • Transthoracic echocardiogram (TTE)
  • N-terminal pro-B-type natriuretic peptide (NT-Pro BNP)

Use of 6-minute walk stress echocardiography has also been proposed as a means of predicting the development of PAH in patients with connective tissue disorders. [27]

Abnormalities on noninvasive tests require confirmation with right heart catheterization, which remains the gold standard for diagnosis of PAH.


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