4A40

ICD-11 MMS 2026-01

Lupus erythematosus

Description

An autoimmune non-organ specific inflammatory disease characterised by the presence of antibodies to DNA, RNA and other components of the nucleus. It has a very variable clinical presentation and course ranging from an acute fulminant life-threatening disorder with involvement of heart, central nervous system and kidneys to an indolent chronic scarring skin disorder.

Exclusions from above levels

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

Child codes

  • 4A40.0 – Systemic lupus erythematosus
  • 4A40.1 – Drug-induced lupus erythematosus
  • 4A40.Y – Other specified lupus erythematosus
  • 4A40.Z – Lupus erythematosus, unspecified

Foundation children elsewhere

  • EB50 – Subacute cutaneous lupus erythematosus
  • EB51 – Chronic cutaneous lupus erythematosus
  • KA07.0 – Neonatal lupus erythematosus

Sibling codes

  • 4A41 – Idiopathic inflammatory myopathy
  • 4A42 – Systemic sclerosis
  • 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

Lupus erythematosus – http://id.who.int/icd/entity/1443317238

Allowed postcoordination axes

Part of: 04: Diseases of the immune system