4A42

ICD-11 MMS 2026-01

Systemic sclerosis

Description

Systemic sclerosis is a systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract.

Inclusions

  • Systemic scleroderma

Excludes

  • EB61.0 – Circumscribed scleroderma

Exclusions from above levels

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

Child codes

  • 4A42.0 – Paediatric onset systemic sclerosis
  • 4A42.1 – Diffuse systemic sclerosis
  • 4A42.2 – Limited systemic sclerosis
  • 4A42.Z – Systemic sclerosis, unspecified

Sibling codes

  • 4A40 – Lupus erythematosus
  • 4A41 – Idiopathic inflammatory myopathy
  • 4A43 – Overlap or undifferentiated nonorgan specific systemic autoimmune disease
  • 4A44 – Vasculitis
  • 4A45 – Antiphospholipid syndrome
  • 4A4Y – Other specified nonorgan specific systemic autoimmune disorders
  • 4A4Z – Nonorgan specific systemic autoimmune disorders, unspecified

Foundation entity

Systemic sclerosis – http://id.who.int/icd/entity/1084365812

Allowed postcoordination axes

Part of: 04: Diseases of the immune system