What is the role of medications in the treatment of mixed connective-tissue disorder (MCTD)?

Updated: Jan 21, 2021
  • Author: Eric L Greidinger, MD; Chief Editor: Herbert S Diamond, MD  more...
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Therapeutic options in mixed connective-tissue disease (MCTD) include the following:

  • Arthritis/arthralgia can often be controlled with prostaglandin inhibitors (nonsteroidal anti-inflammatory drugs (NSAIDs) or omega-3 fatty acids); antimalarials (hydroxychloroquine); and, if needed, infrequent oral or intra-articular corticosteroid courses, typically at low dose.
  • Disease-modifying antirheumatic drug (DMARD) therapy is reserved for more refractory synovitis, particularly if showing features of erosive/deforming risk.
  • Proton pump inhibitors can control esophageal reflux symptoms.
  • In patients with Raynaud phenomenon, calcium channel blocking agents are used.
  • Phosphodiesterase inhibitors, endothelin receptor antagonists, or prostaglandins can be used for pulmonary hypertension. 
  • In some cases, pulmonary hypertension in MCTD may also respond to aggressive immunosuppression, similar to that used in interstitial lung disease therapy. [35]
  • MCTD-related interstitial lung disease is conventionally treated with corticosteroids, in combination with steroid-sparing agents such as cyclophosphamide, azathioprine, or mycophenolate. [36]  The INBUILD trial, which included patients with MCTD, reported that treatment with the antifibrotic tyrosine kinase inhibitor nintedanib can significantly slow the annual rate of decline in forced vital capacity (FVC) in patients with progressive fibrosing interstitial lung diseases. [37]

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