Cancer associated retinopathy

Short description: Autoimmune eye disease

Cancer Associated Retinopathy

Specialty| Ophthalmology, optometry
Complications| Blindness[1]
Diagnostic method| Eye examination
Differential diagnosis| Retinitis pigmentosa
Treatment| Immunosuppression

Cancer Associated Retinopathy (CAR) also known as Carcinoma Associated Retinopathy is an immune-mediated disease affecting the retina of the eye. It is a paraneoplastic type autoimmune retinopathy associated with cancer that can cause blindness. It can be seen in association with various types of cancers. It can be treated with a combination of chemotherapy and immunosuppression.

## Contents

* 1 Pathophysiology
* 2 Prevalence
* 3 Signs and symptoms
* 4 Diagnosis
* 5 Treatment
* 6 History
* 7 References

## Pathophysiology

It is a paraneoplastic type of autoimmune retinopathy.[2] It may be seen in association with various cancers including non-small cell lung cancer, breast cancer, gynacological cancers, tumors of the hematopoietic and lymphoid tissues, basal cell carcinoma, colon cancer, kidney cancer, prostate cancer and pancreatic cancer.[1]

CAR results from the interaction between retinal antigen expression in cancer tissues and their systemic immune response. These antigens trigger an autoimmune response within the host to form antibodies that cross-react with the retinal antigen. This ultimately leads to retinal degeneration and apoptosis. Recoverin is the most common antigen associated with CAR.[3]

## Prevalence

The prevalence of CAR among cancer patients is thought to be 10%–15%.[4]

## Signs and symptoms

Patients may complain loss of vision, black spots in the field of vision (scotoma), night blindness, prolonged glare after light exposure, prolonged dark adaptation and defects in colour vision (dyschromatopsia).[5] Major signs include circulating anti-retinal antibodies along with loss of the outer retinal layer including the ellipsoid layer and photoreceptor cells, abnormalities in ERG, fundus autofluorescence and visual field defectss.[5]

## Diagnosis

CAR is suspected in cancer patients who present with unexplained visual loss, scotomas and an abnormal ERG.[6] Antiretinal antibodies can be detected using Western blot test, immunohistochemistry test or ELISA testing. But the diagnosis is challenging as the signs and symptoms overlaps with many other diseases affecting the retina.[7]

## Treatment

Long-term systemic Immunosuppressive therapy is the main treatment of cancer-associated retinopathy.[5] It can be treated with a combination of chemotherapy and immunosuppression.[2] Although tumor removal and cancer regression may result in a decrease in circulating autoantibodies, this does not influence CAR progression.[8]

Systematic immunosuppressive medications used in the treatment include corticosteroids, cyclosporin, azathioprine and alemtuzumab.[5] Intravenous immunoglobulin injections have several advantages including neutralization of autoantibodies.[5] Various monoclonal antibodies are also used to treat CAR.[1]

## History

In 1976, cancer-related vision loss and photoreceptor dysfunction were first described by Sawyer et al.[2]

## References

1. ↑ 1.0 1.1 1.2 Singh, Dheerendra; Tripathy, Koushik (2022), "Cancer Associated Retinopathy", StatPearls (Treasure Island (FL): StatPearls Publishing), PMID 35201711, http://www.ncbi.nlm.nih.gov/books/NBK578183/, retrieved 2023-02-16
2. ↑ 2.0 2.1 2.2 Grange, Landon; Dalal, Monica; Nussenblatt, Robert B.; Sen, H. Nida (February 2014). "Autoimmune Retinopathy". American Journal of Ophthalmology 157 (2): 266–272.e1. doi:10.1016/j.ajo.2013.09.019. ISSN 0002-9394. PMID 24315290.
3. ↑ Dy, Irene; Chintapatla, Rangaswamy; Preeshagul, Isabel; Becker, Daniel (2013-11-01). "Treatment of Cancer-Associated Retinopathy With Rituximab" (in en-US). Journal of the National Comprehensive Cancer Network 11 (11): 1320–1324. doi:10.6004/jnccn.2013.0156. ISSN 1540-1405. PMID 24225966. https://jnccn.org/view/journals/jnccn/11/11/article-p1320.xml.
4. ↑ Dimitriou, Eleni; Chatzirallis, Alexandros; Katopodis, Sokratis; Theodossiadis, George; Theodossiadis, Panagiotis; Chatziralli, Irini (2021-05-01). "Cancer-associated retinopathy 4 years after surgery for ovarian cancer" (in en). Oman Journal of Ophthalmology 14 (2): 108–111. doi:10.4103/ojo.ojo_352_20. ISSN 0974-620X. PMID 34345145. PMC 8300284. https://www.ojoonline.org/article.asp?issn=0974-620X;year=2021;volume=14;issue=2;spage=108;epage=111;aulast=Dimitriou;type=0.
5. ↑ 5.0 5.1 5.2 5.3 5.4 "Cancer Associated Retinopathy - EyeWiki". https://eyewiki.aao.org/Cancer_Associated_Retinopathy.
6. ↑ Goetgebuer, Griet; Kestelyn-Stevens, A.-M.; Laey, J.; Kestelyn, P.; Leroy, B. (2007). "Cancer-associated retinopathy (CAR) with electronegative ERG: a case report" (in en). Documenta Ophthalmologica 116: 49–55. doi:10.1007/s10633-007-9074-9. PMID 17721792. https://www.semanticscholar.org/paper/Cancer-associated-retinopathy-(CAR)-with-ERG:-a-Goetgebuer-Kestelyn-Stevens/7aa27efff08a08ce18f654991b04a8b3932ab8fa.
7. ↑ "Diagnosis and Management of Autoimmune Retinopathy" (in en). 2017-09-01. https://www.aao.org/eyenet/article/diagnosis-and-management-of-autoimmune-retinopathy.
8. ↑ "Cancer associated retinopathy" (in en). https://www.eyenews.uk.com/education/trainees/post/cancer-associated-retinopathy.

* v
* t
* e

* Diseases of the human eye (H00–H59
* 360–379)

Adnexa

| Eyelid|

| Inflammation|

* Stye
* Chalazion
* Blepharitis

|

* Entropion
* Ectropion
* Lagophthalmos
* Blepharochalasis
* Ptosis
* Blepharophimosis
* Xanthelasma

Eyelash|

* Trichiasis
* Madarosis

Lacrimal apparatus|

* Dacryoadenitis
* Epiphora
* Dacryocystitis
* Xerophthalmia

Orbit|

* Exophthalmos
* Enophthalmos
* Orbital cellulitis
* Orbital lymphoma
* Periorbital cellulitis

Conjunctiva|

* Conjunctivitis
* allergic
* Pterygium
* Pinguecula
* Subconjunctival hemorrhage

Globe

| Fibrous tunic|

| Sclera|

* Scleritis
* Episcleritis

|

Cornea|

* Keratitis
* herpetic
* acanthamoebic
* fungal
* Corneal ulcer
* Photokeratitis
* Thygeson's superficial punctate keratopathy
* Corneal dystrophy
* Fuchs'
* Meesmann
* Corneal ectasia
* Keratoconus
* Pellucid marginal degeneration
* Keratoglobus
* Terrien's marginal degeneration
* Post-LASIK ectasia
* Keratoconjunctivitis
* sicca
* Corneal neovascularization
* Kayser–Fleischer ring
* Haab's striae
* Arcus senilis
* Band keratopathy

Vascular tunic|

* Iris
* Ciliary body

* Uveitis
* Intermediate uveitis
* Hyphema
* Rubeosis iridis
* Persistent pupillary membrane
* Iridodialysis
* Synechia

|

Choroid|

* Choroideremia
* Choroiditis
* Chorioretinitis

Lens|

* Cataract
* Congenital cataract
* Childhood cataract
* Aphakia
* Ectopia lentis

Retina|

* Retinitis
* Chorioretinitis
* Cytomegalovirus retinitis
* Retinal detachment
* Retinoschisis
* Ocular ischemic syndrome / Central retinal vein occlusion
* Central retinal artery occlusion
* Branch retinal artery occlusion
* Retinopathy
* diabetic
* hypertensive
* Purtscher's
* of prematurity
* Bietti's crystalline dystrophy
* Coats' disease
* Macular degeneration
* Retinitis pigmentosa
* Retinal haemorrhage
* Central serous retinopathy
* Macular edema
* Epiretinal membrane (Macular pucker)
* Vitelliform macular dystrophy
* Leber's congenital amaurosis
* Birdshot chorioretinopathy

Other|

* Glaucoma / Ocular hypertension / Primary juvenile glaucoma
* Floater
* Leber's hereditary optic neuropathy
* Red eye
* Globe rupture
* Keratomycosis
* Phthisis bulbi
* Persistent fetal vasculature / Persistent hyperplastic primary vitreous
* Persistent tunica vasculosa lentis
* Familial exudative vitreoretinopathy

Pathways

| Optic nerve
Optic disc|

* Optic neuritis
* optic papillitis
* Papilledema
* Foster Kennedy syndrome
* Optic atrophy
* Optic disc drusen

Optic neuropathy|

* Ischemic
* anterior (AION)
* posterior (PION)
* Kjer's
* Leber's hereditary
* Toxic and nutritional

Strabismus
Extraocular muscles
Binocular vision
Accommodation|

| Paralytic strabismus|

* Ophthalmoparesis
* Chronic progressive external ophthalmoplegia
* Kearns–Sayre syndrome

palsies|

* Oculomotor (III)
* Fourth-nerve (IV)
* Sixth-nerve (VI)

Other strabismus|

* Esotropia / Exotropia
* Hypertropia
* Heterophoria
* Esophoria
* Exophoria
* Cyclotropia
* Brown's syndrome
* Duane syndrome

Other binocular|

* Conjugate gaze palsy
* Convergence insufficiency
* Internuclear ophthalmoplegia
* One and a half syndrome

Refraction|

* Refractive error
* Hyperopia
* Myopia
* Astigmatism
* Anisometropia / Aniseikonia
* Presbyopia

Vision disorders
Blindness|

* Amblyopia
* Leber's congenital amaurosis
* Diplopia
* Scotoma
* Color blindness
* Achromatopsia
* Dichromacy
* Monochromacy
* Nyctalopia
* Oguchi disease
* Blindness / Vision loss / Visual impairment

Anopsia|

* Hemianopsia
* binasal
* bitemporal
* homonymous
* Quadrantanopia

subjective|

* Asthenopia
* Hemeralopia
* Photophobia
* Scintillating scotoma

Pupil|

* Anisocoria
* Argyll Robertson pupil
* Marcus Gunn pupil
* Adie syndrome
* Miosis
* Mydriasis
* Cycloplegia
* Parinaud's syndrome

Other|

* Nystagmus
* Childhood blindness

Infections

* Trachoma
* Onchocerciasis

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