4A43.3

ICD-11 MMS 2026-01

Mixed connective tissue disease

Description

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.

Index terms

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

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A43.0 – IgG4 related disease
  • 4A43.1 – Mikulicz disease
  • 4A43.2 – Sjögren syndrome
  • 4A43.4 – Diffuse eosinophilic fasciitis
  • 4A43.Y – Other specified overlap non-organ specific systemic autoimmune disease
  • 4A43.Z – Undifferentiated non-organ specific systemic autoimmune disease

Foundation entity

Mixed connective tissue disease – http://id.who.int/icd/entity/891652224

Part of: 4A43: Overlap or undifferentiated nonorgan specific systemic autoimmune disease › 04: Diseases of the immune system