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
Requirements:
- Serological
- At least 3 clinical features
- 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
- Reynaud’s Phenomenon
- 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.
References:
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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]
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Alarcon-Segovia D. Mixed connective tissue disease and overlap syndromes. Clin Dermatol. 1994 Apr-Jun;12(2):309-16. [Medline]
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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]
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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
