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4A43.3 Mixed connective tissue disease

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Mixed connective tissue disease is an overlapping syndrome combining features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with the presence of autoantibodies to U1-ribonucleoprotein. Raynaud’s phenomenon is seen in nearly all patients and pulmonary arterial hypertension is the most common cause of death in MCTD patients.

synonyms

• Mixed connective tissue disease
• Sharp syndrome
• MCTD - [mixed connective tissue disease]
• Paediatric-onset mixed connective tissue disease
• Paediatric-onset Sharp syndrome
• Paediatric-onset MCTD - [mixed connective tissue disease]

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