What is the prognosis 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

Most patients with MCTD have a favorable outcome. Cases of MCTD with typical clinical or serologic features occasionally evolve into scleroderma, SLE, or another rheumatic disease.

Pulmonary hypertension is the most common disease-associated cause of death. Careful monitoring and aggressive treatment may improve the outcome of pulmonary hypertension. 

A long-term observational nationwide cohort study from Norway found that interstitial lung disease (ILD) was present in 41% of MCTD patients and progressed in 19% of patients across the observation period of a mean of 6.4 years. [18] The following were the strongest predictors of ILD progression:

  • Male sex  (hazard ratio [HR] = 4.0, 95% confidence index [CI]: 1.4, 11.5; P = 0.011)
  • Presence of anti-ro52 antibodies (HR = 3.5, 95% CI: 1.2, 10.2; P = 0.023)
  • Elevated anti-RNP titer (HR 1.5, 95% CI: 1.1, 2.0; P = 0.008)
  • Absence of arthritis (HR = 0.2, 95% CI: 0.1, 0.6; P = 0.004) 

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