ICD-11 classes
08 Diseases of the nervous system
Other disorders of the nervous system
8E4A — Paraneoplastic or autoimmune disorders of the nervous system
8E4A.0 — Paraneoplastic or autoimmune disorders of the central...
9B71.4 — Paraneoplastic retinopathy

ICD-11 9B71.4 — Paraneoplastic retinopathy

Paraneoplastic retinopathies results 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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 9B71.4 contains 3 clarifying diagnoses:

  1. 9B71.40 — Melanoma associated retinopathy
  2. 9B71.4Y — Other specified paraneoplastic retinopathy
  3. 9B71.4Z — Paraneoplastic retinopathy, unspecified

The diagnosis is coded elsewhere:

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