4A43.2

ICD-11 MMS 2026-01

Sjögren syndrome

Description

Sjögren syndrome (SS) is a slowly progressive, systemic inflammatory autoimmune disease affecting primarily the exocrine glands. Lymphocytic infiltrates replace functional epithelium, leading to oral and ocular dryness. Characteristic autoantibodies (e.g., anti-Ro/SS-A and/or anti-La/SS-B) are produced. The disorder can occur alone (it is then known as “primary SS”) or in association with another autoimmune disease (it is then known as “secondary SS”).

Exclusions from above levels

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

Child codes

  • 4A43.20 – Primary Sjögren syndrome
  • 4A43.21 – Secondary Sjögren syndrome
  • 4A43.22 – Paediatric onset Sjögren syndrome
  • 4A43.2Y – Other specified sjögren syndrome
  • 4A43.2Z – Sjögren syndrome, unspecified

Foundation children elsewhere

  • 9A79 – Keratoconjunctivitis sicca

Sibling codes

  • 4A43.0 – IgG4 related disease
  • 4A43.1 – Mikulicz disease
  • 4A43.3 – Mixed connective tissue disease
  • 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

Sjögren syndrome – http://id.who.int/icd/entity/1923044320

Allowed postcoordination axes

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