ICD-11 code : 9B71.4;
Preferred Label : Paraneoplastic retinopathy;
ICD-11 definition : Paraneoplastic retinopathies results from a targeted attack on the retina due to a
tumor 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, color 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 recognized:
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.;
ICD-11 synonym : Cancer associated retinopathy;
ICD-11 "other" category code : 9B71.4Y;
ICD-11 "unspecified" category code : 9B71.4Z;
Origin ID : 1216073790;
Automatic exact mappings (from CISMeF team)
Paraneoplastic retinopathies results from a targeted attack on the retina due to a
tumor 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, color 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 recognized:
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.
