Mixed connective tissue disease natural history, complications and prognosis

If the patients with mixed connective tissue disease left untreated, approximately 35% of patients may progress to develop lung fibrosis. Common complications of MCTD include polyarthritis, Raynaud’s phenomenon, interstitial lung disease, esophageal dysmotility, sclerodactyly, polymyositis, and pulmonary hypertension. Major causes of morbidity in long-term MCTD patients include sclerodactyly, diffuse sclerosis, pulmonary arterial hypertension, and nervous system disease. The presence of pulmonary involvement is associated with worst prognosis and high mortality rate.

• The symptoms of mixed connective tissue disease usually develop in the first decades of life, and presents with symptoms of other rheumatologic diseases such as SLE, systemic sclerosis, polymyositis, or rheumatoid arthritis.^[1]

• If left untreated, approximately 35% of patients with mixed connective tissue disease may progress to develop lung fibrosis.^[2]

• Common complications of MCTD include:^[3][4][5]
  • Polyarthritis
  • Raynaud’s phenomenon
  • Puffy fingers (sausage digits)
  • Interstitial lung disease
  • Esophageal dysmotility
  • Sclerodactyly
  • Polymyositis
  • Pulmonary hypertension

• In patients with MCTD the prognosis is relatively good.^[6]
• In MCTD, prognosis depends on the type of organ involvement.^[7]
• Major causes of morbidity in long-term MCTD patients include:^[8]
  • Sclerodactyly
  • Diffuse sclerosis
  • Pulmonary arterial hypertension
  • Nervous system disease
• The presence of pulmonary involvement is associated with worst prognosis and high mortality rate.^[9]

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