Mixed Connective-Tissue Disease Follow-up

Updated: Sep 29, 2017
  • Author: Eric L Greidinger, MD; Chief Editor: Herbert S Diamond, MD  more...
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Follow-up

Further Outpatient Care

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. [23] 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. [24]

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

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Further Inpatient Care

See the list below:

  • Patients with mixed connective-tissue disease (MCTD) may require admission pending assessment for suspected infection or complications related to disease or treatment.
  • Admit patients to appropriate service with rheumatology care, if available. Obtain subspecialty consultations as indicated.
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Patient Education

See the list below:

  • Education about MCTD and its treatment is essential.
  • Active participation in the decision-making process empowers patients in their own care.
  • Education about disease decreases the risk of patients developing learned helplessness and improves functional outcomes.
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