" /> Mixed connective tissue disease - CISMeF





ICD-11 code : 4A43.3;

Preferred Label : Mixed connective tissue disease;

ICD-11 definition : 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.;

ICD-11 synonym : MCTD - [mixed connective tissue disease]; Sharp syndrome;

ICD-11 acronym : MCTD;

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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.

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28/07/2025


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