Unifying Concepts

Diagnostic Criteria for Mixed Connective Tissue Disease (MCTD)

Mixed connective tissue disease (MCTD) has signs and symptoms of a combination of disorders, primarily lupus, scleroderma, and polymyositis. Many people with this uncommon disease also have Sjogren’s syndrome. For this reason, MCTD is sometimes called an overlap disease.

Alarcon-Segovia Diagnostic Criteria for Mixed Connective Tissue Disease (MCTD)

1. Serological criteria: Positive anti U1 RNP at haemagglutination titer >1:1600

2. Clinical criteria:

  • Oedema of hands
  • Synovitis
  • Myositis
  • Reynaud’s
  • Acrosclerosis


  1. Serological
  2. At least 3 clinical features
  3. Association of hand oedema, Reynaud’s and acrosclerosis requires at least one other feature

Kusukawa Diagnostic Criteria for Mixed Connective Tissue Disease (MCTD)

Common Symptoms

  1. Reynaud’s Phenomenon
  2. Swollen fingers or hands

Presence of Anti U1 RNP

Mixed findings

A. Systemic lupus erythematosus (SLE) like

  • Polyarthritis
  • Pericarditis/pleuritis
  • Lymphadenopathy
  • Facial erithema
  • Leucopenia/thrombocytopenia

B. Scleroderma like

  • Sclerodactyly
  • Pulmonary fibrosis
  • Esophageal dysmotility

C. Polymyositis like

  • Muscle weakness
  • High creatine phosphokinase (CPK)
  • Myophatic electromyogram (EMG)

Requirement for diagnosis: At least one common symptom, with positive U1 RNP antibodies and one or more findings in at least two of the three categories A, B, and C.



  1. Alarcon-Segovia D, Cardiel MH. Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. J Rheumatol. 1989 Mar;16(3):328-34. [Medline]

  2. Alarcon-Segovia D. Mixed connective tissue disease and overlap syndromes. Clin Dermatol. 1994 Apr-Jun;12(2):309-16. [Medline]

  3. Tiddens HA, van der Net JJ, de Graeff-Meeder ER, Fiselier TJ, de Rooij DJ, van Luijk WH, Herzberger R, van Suijlekom LW, van Venrooij WJ, Zegers BJ, et al. Juvenile-onset mixed connective tissue disease: longitudinal follow-up. J Pediatr. 1993 Feb;122(2):191-7. [Medline]

  4. Haroon N, Nisha RS, Chandran V, Bharadwaj A. Pulmonary hypertension not a major feature of early mixed connective tissue disease: A prospective clinicoserological study. J Postgrad Med. 2005 Apr-Jun;51(2):104-8. [Medline]


Created: Mar 24, 2006

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