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Mixed connective tissue disease.

P J W Venables1

  • 1Kennedy Institute, Imperial College, London, UK. p.venables@imperial.ac.uk

Lupus
|April 26, 2006
PubMed
Summary

Mixed connective tissue disease (MCTD) is a complex syndrome defined by antibodies to U1RNP. Long-term studies reveal it can evolve into SLE or systemic sclerosis, with significant mortality risks.

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Area of Science:

  • Rheumatology
  • Immunology
  • Pathology

Background:

  • Mixed connective tissue disease (MCTD) presents with overlapping features of systemic sclerosis, systemic lupus erythematosus (SLE), and polymyositis.
  • It is characterized by antibodies to the U1 ribonuclear protein (U1RNP) component of the spliceosome.
  • MCTD was the first rheumatic disease syndrome defined by a serologic test.

Purpose of the Study:

  • To review the pathological, immunogenetic, and clinical features of MCTD.
  • To address the ongoing debate regarding MCTD as a distinct clinical entity.
  • To evaluate the long-term prognosis and evolution of MCTD.

Main Methods:

  • Review of pathological, immunogenetic, and clinical data.
  • Analysis of long-term follow-up studies.
  • Examination of the evolution of clinical features associated with anti-U1RNP antibodies.

Main Results:

  • MCTD exhibits a high frequency of Raynaud's syndrome, swollen hands, sclerodactyly, arthritis, polymyositis, and interstitial lung disease.
  • Long-term follow-up indicates MCTD can evolve into SLE or systemic sclerosis.
  • Pulmonary hypertension and scleroderma renal crisis are identified as significant causes of mortality.

Conclusions:

  • The debate on whether MCTD is a distinct clinical entity remains unresolved.
  • The initial perception of a good prognosis for MCTD has been revised by long-term data.
  • MCTD remains a useful clinical concept, though its classification as a 'disease' awaits further etiological evidence.

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