9B71.4

ICD-11 MMS 2026-01

Paraneoplastic retinopathy

Description

Paraneoplastic retinopathies result from a targeted attack on the retina due to a tumour immune response initiated by onco-neural antigens derived from systemic cancer. Patients usually present after cancer diagnosis with progressive visual dimming and photopsias but dysfunction of rods (impaired dark adaption and peripheral vision loss) and cones (decreased visual acuity, colour dysfunction, photosensitivity and glare) may also occur. Symptoms are often worse than clinical signs. Other causes of retinopathy should be excluded. Multiple anti-retinal autoantibodies (e.g. anti-recoverin antibodies) are described although their significance is uncertain. Two major subsets are recognised: cancer-associated retinopathy (most commonly small-cell lung cancer) and melanoma-associated retinopathy. Associated neural autoantibodies include: CRMP5 (anti-CV2) (collapsin response mediator protein 5 - anti CV2); anti-recoverin autoantibodies; and alpha-enolase autoantibodies.

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 8A21.0 – Posterior cortical atrophy

Child codes

  • 9B71.40 – Melanoma associated retinopathy
  • 9B71.4Y – Other specified paraneoplastic retinopathy
  • 9B71.4Z – Paraneoplastic retinopathy, unspecified

Sibling codes

  • 9B71.0 – Diabetic retinopathy
  • 9B71.1 – Hypertensive retinopathy
  • 9B71.2 – Radiation retinopathy
  • 9B71.3 – Retinopathy of prematurity
  • 9B71.5 – Autoimmune retinopathy
  • 9B71.Y – Other specified retinopathy
  • 9B71.Z – Retinopathy, unspecified

Foundation entity

Paraneoplastic retinopathy – http://id.who.int/icd/entity/1216073790

Allowed postcoordination axes

Part of: 9B71: Retinopathy › 09: Diseases of the visual system