Mixed Connective-Tissue Disease (MCTD) Treatment & Management

Updated: Dec 22, 2022
  • Author: Eric L Greidinger, MD; Chief Editor: Herbert S Diamond, MD  more...
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Approach Considerations

The overall goals of therapy for mixed connective-tissue disease (MCTD) are to control symptoms, to maintain function, and to reduce the risk of future disease consequences. Medical therapy targets control of disease activity in general and management of specific organ involvement (see Medication), while monitoring for and mitigating the risks of complications either of the condition itself (eg, pulmonary hypertension, interstitial lung disease) or of its treatment (eg, infection) (see Follow-up).

Patients with MCTD may require hospital admission while undergoing assessment for suspected infection or complications related to disease or treatment.  Admit patients to the appropriate service with rheumatology care, if available. Obtain subspecialty consultations as indicated.




Whenever possible, a rheumatologist experienced in diagnosis and treatment of the disease should co-manage all patients with mixed connective-tissue disease (MCTD). Consultation with other specialists or subspecialists may be indicated for the evaluation and/or treatment of specific aspects of disease, such as pulmonary hypertension, interstitial lung disease, gastroesophageal reflux, or acute ischemia due to Raynaud's phenomenon.


Diet and Activity

Aggressive cold avoidance may help to reduce the risk of flares of Raynaud's Phenomenon.

Patients with hypertension, esophageal reflux, malabsorption, or other sclerodermatous-type bowel involvement may need special consideration.

Because atherosclerotic heart disease remains a major risk in all patients, advocate a heart-healthy diet. However, no specific dietary manipulations have been demonstrated to be effective in treating MCTD.

Convincing data support the value of an active lifestyle and an exercise program tailored to the needs of patients with arthritis of various types. This approach also appears to be appropriate in MCTD.



Given that other inflammatory rheumatic diseases—including rheumatoid arthritis, lupus, and scleroderma—are reported to be independent risk factors for the development of atherosclerosis and related conditions, patients with MCTD may also be at increased risk for atherosclerosis. As in those other conditions (and modeled after recommendations developed for diabetes mellitus), more aggressive targets for blood pressure and cholesterol control may be appropriate.


Long-Term Monitoring

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. [36]  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) tests

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. [37]

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