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
  • Raynaud´s
  • Acrosclerosis


  1. Serological
  2. At least 3 clinical features
  3. Association of hand oedema, Raynaud´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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