Cancer-associated Retinopathy Research Articles

Case Report on Cancer-associated Retinopathy (CAR) with Disease Stabilization on Immunosuppressive Therapy (P1-14.019)

Case Report on Cancer-associated Retinopathy (CAR) with Disease Stabilization on Immunosuppressive Therapy (P1-14.019)

Paraneoplastic panuveitis: a case report revealing non-small cell lung cancer with asymmetric steroid response

Intraocular inflammation can affect nearly all structures of the eye from anterior to posterior. Rarely, intraocular inflammation is caused by a paraneoplastic syndrome (PNS). In cancer-associated retinopathy (CAR), the symptoms, which typically affect both eyes, include severe loss of vision, photosensitivity, ring scotoma, and visual field defects [1]. The following case outlines a diagnostic challenge with an initial clinical presentation of photopsias and isolated dense vitritis.

Successful Treatment of Clinically Diagnosed Cancer-Associated Retinopathy with Intravitreal Dexamethasone Implant Followed by 0.18 mg Fluocinolone Implant without Systemic Immunosuppression.

To report a case of clinically diagnosed cancer-associated retinopathy (CAR) successfully treated with intravitreal corticosteroid implants without systemic immunosuppression. Case report with multimodal imaging. An 80-year-old man without known systemic malignancy presented with debilitating shimmering, hemeralopia and rapidly progressive bilateral vision loss following uncomplicated cataract surgery. Mild vitritis, extensive photoreceptor loss, mottling of retinal pigmentary epithelium (RPE), and mild vascular attenuation were found in both eyes. Full field electroretinogram (ffERG) showed severe bilateral rod-cone dysfunction. Infectious etiologies and vitreoretinal lymphoma were ruled out. During cancer workup, intravitreal corticosteroid treatment was offered. Significant anatomical improvement with reconstitution of ellipsoid zone, improved RPE irregularities and functional improvement, were observed 3 weeks after bilateral intravitreal dexamethasone implants (Ozurdex). 2 months later, patient received bilateral intravitreal 0.18mg fluocinolone acetonide implants (YUTIQ). Later, a colonic adenocarcinoma was found (pathologic stage pT3 pN0). Patient recovered well from surgery and no chemotherapy was needed. 9 months since bilateral intravitreal fluocinolone acetonide implants (11 months since bilateral intravitreal dexamethasone implants), best corrected vision maintained at 20/25-2 OD, 20/20 OS without ongoing treatments. Bilateral reconstitution of ellipsoid zones and nearly resolution of RPE irregularities remained stable. Repeat ffERG demonstrated improved cone response OS and stable diminished rod response OU. Patient reports resolution of ocular symptoms. The sustained improvements with intravitreal corticosteroid monotherapy suggest potential advantages using local therapy over systemic treatment. Long term follow-up is warranted. Further research is needed to evaluate the efficacy of using 0.18mg fluocinolone implant (YUTIQ) to treat CAR.

Cancer-associated retinopathy as an initial presentation of gynecologic small-cell carcinoma

Abstract: A 56-year-old female presented with bilateral progressive blurred vision over 1 month. She has no known malignancy before her initial visit to our ophthalmologic clinic. Her best-corrected visual acuity decreased to hand motion from 30 cm in both eyes. Optical coherence tomography exhibited parafoveal thinning of outer retinal layers bilaterally. Fluorescein angiography and indocyanine green angiography disclosed hypofluorescent spots in late phase in both eyes. The suspicion of cancer-associated retinopathy (CAR) prompted us to investigate and refer for further systemic disease including occult malignancy. The patient was diagnosed with small-cell carcinoma of the endometrium or cervix, which is an extremely rare and aggressive neuroendocrine tumor. The patient was treated with oral prednisone with improved visual acuity. The patient expired from sepsis 2 months after her initial visit to our ophthalmologic clinic. In selected cases, CAR may present before the diagnosis of primary cancer. It is essential to recognize its ophthalmic manifestation for early discovery of primary malignancy.

Review of Autoimmune Retinopathy and Its Association with Melanoma and Other Malignancies

Autoimmune Retinopathy (AIR) is an immune-mediated degenerative retinal disorder affecting retinal cell function leading to progressive vision loss. This review aims to quantify documented AIR cases in the literature; and elucidate common visual symptoms, AIR and cancer diagnosis chronology and autoantibodies’ role in AIR pathophysiology. A literature search extracted 58 Melanoma-Associated Retinopathy (MAR) and 76 Cancer-Associated Retinopathy (CAR) cases; CAR primarily consisted of lung, endometrial, ovarian and breast cancer. The majority of MAR cases presented with a melanoma diagnosis preceding onset of AIR symptoms, while the majority of CAR cases presented with AIR symptoms preceding the diagnosis of malignancy. MAR patients experienced nyctalopia and photopsias while CAR patients experienced vision loss, photopsias and nyctalopia. Anti-recoverin is the most well-established antibody implicated in AIR pathogenicity. However, autoantibodies to α-enolase, aldolase A and C, transducin-α, carbonic anhydrase II, arrestin, GAPDH and Transient Receptor Potential cation channel, subfamily M, member 1 (TRPM1) are also key components of retinal degeneration. AIR is likely caused by antibodies-targeting retinal antigens aberrantly expressed in cancer cells-penetrating the blood-retinal barrier and cross-reacting with retinal cell antigens, inducing retinal pathology. It may be appropriate to include AIR vision panel screening as standard of care for individuals with personal/family history or signs/symptoms of retinopathy or cancers for early detection and intervention.

Cancer-Associated Retinopathy (CAR) And Melanoma-Associated Retinopathy (MAR)

Cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR) are rare paraneoplastic syndromes in which antibodies are directed against retinal antigens, independent of primary tumor or metastasis, that can cause vision loss. The fact that these antigens differ according to different types of cancer causes the developing retinopathy and accordingly the symptoms to be heterogeneous and causing difficulties in diagnosis and treatment. The literature on this subject is limited with few reported case reports and case series. The lack of controlled clinical studies also makes it difficult for clinicians to manage these cases. Paraneoplastic syndromes are important because they can diagnose undiagnosed malignancies or indicate recurrence of treated malignancies. The appearance of the symptoms of CAR and MAR is of great importance for the prognosis of cancer, as it may precede cancer findings. In addition, with early diagnosis, irreversible retinal damage will be minimal. In this review, the pathophysiology, clinical findings and treatments of CAR and MAR are emphasized.

Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Our purpose is to describe blue-light fundus autofluorescence (BAF) features of inflammatory diseases of the outer retina characterised by photoreceptor damage. BAF from patients diagnosed with secondary and primary inflammatory photoreceptor damage were retrospectively analyzed and compared to other imaging modalities including fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT). Multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and acute syphilitic posterior placoid chorioretinitis (ASPPC), all cases corresponding to secondary photoreceptor diseases caused by inflammatory choriocapillaris nonperfusion, were included and compared to primary photoreceptor disease entities, including acute zonal occult outer retinopathy (AZOOR) and cancer-associated retinopathy (CAR). Both groups showed increased BAFs of variable intensity. In severe cases of APMPPE and ASPPC, BAF also showed hypoautofluorescent areas. In group 1 (secondary diseases) BAF hyperautofluorescent areas were associated with colocalized ICGA hypofluorescent areas, indicating choriocapillaris nonperfusion; whereas in group 2 (primary diseases), no ICGA signs were detected. The associated colocalized areas of hypofluorescence on ICGA in the first group, which were absent in the second group, were crucial to allow the differentiation between primary (photoreceptoritis) and secondary (choriocapillaritis) photoreceptor diseases. BAF patterns in inflammatory diseases of the outer retina can give relevant information on the photoreceptor and RPE involvement, with ICGA being crucial to detect concurring choriocapillaris damage and differentiating the two pathologies.

Management of autoimmune retinopathy treated with intravitreal dexamethasone implant.

The purpose of this study is to evaluate clinical outcomes of autoimmune retinopathy (AIR) in the patients treated with intravitreal dexamethasone implant (IDI). Twenty-one eyes of 11 AIR patients treated with at least 1 injection of IDI were retrospectively reviewed. Clinical outcomes before and after treatment, including best corrected visual acuity (BCVA), optic coherence tomography (OCT), fundus autofluorescence (FAF), full-field electroretinography (ff-ERG), and visual field (VF) at last visit within 6 and/or 12months, were recorded. Among all the patients, 3 had cancer-associated retinopathy (CAR) and 8 had non-paraneoplastic-AIR (npAIR) with mean followed up of 8.52 ± 3.03months (range 4-12months). All patients achieved improved or stable BCVA within 6 and/or 12months after the treatment. Cystoid macular edema (CME) in 2 eyes and significant retinal inflammation in 4 eyes were markedly resolved after single injection. Central retinal thickness (CFT) in all eyes without CME, ellipsoid zone (EZ) on OCT in 71.4% of eyes, ERG response in 55% of eyes, and VF in 50% of eyes were stable or improved within 6months after treatment. At last visit within 12months, both BCVA and CFT remained stable in the eyes treated with either single or repeated IDI; however, progression of EZ loss and damage of ERG response occurred in some patients with single IDI. Clinical outcomes, including BCVA and parameters of OCT, ERG, and VF, were stable or improved after IDI in a majority of AIR patients. Local treatment of AIR with IDI was a good option to initiate the management or an alternative for the patients' refractory to the systemic therapy but with limited side effect.

Paraneoplastic syndromes in ophthalmology

Paraneoplastic syndromes consist on systemic manifestations associated with certain cancers which are not a direct consequence of tumor invasion or its metastases. It is known that autoimmunity and autoantibody synthesis play an important role in its pathophysiology due to a process of molecular mimicry. Paraneoplastic syndromes in ophthalmology are rare, but it is important to recognize them clinically because in some cases symptoms preceded the diagnosis of an underlying neoplasia. Most frequently involved cancer is small cell lung carcinoma, but there is also a relationship with other tumor etiologies such as thymoma, gynecological tumors or neuroblastoma in children. Paraneoplastic syndromes with ocular involvement can be divided into those that affect the afferent visual pathway, such as cancer-associated retinopathy, melanoma-associated retinopathy, or paraneoplastic optic neuropathy; and the ones that affect the efferent visual pathway, such as bilateral tonic pupils, Myasthenia Gravis, Lambert-Eaton syndrome or paraneoplastic cerebellar degeneration. The presence of autoantibodies is helpful in clinical practice but negativity does not exclude this diagnosis. Although evolution and prognosis is linked to primary disease, in some cases specific treatment, usually immunosuppressive therapy, can help improving patients quality of life.

Neuro-ophthalmic manifestations of cancer: a narrative review.

Neuro-ophthalmic manifestations of cancer are vast and early recognition of a serious ocular condition due to either cancer or its therapy is important for both vision preservation as well as providing valuable treatment and prognostic information regarding the underlying malignancy. This review focuses on direct and indirect effects of cancer on the eye and its adnexa, hematologic malignancy, complications of traditional and novel oncologic therapies, and paraneoplastic syndromes as they relate to the eye as these disorders can lead to potentially devastating or irreversible vision loss. PubMed was searched primarily for the following topics: optic nerve infiltration, primary vitreoretinal lymphoma (PVRL), ocular paraneoplastic disorders, and ophthalmic complications of cancer therapeutics. Literature was selected based on historical significance and landmark studies (e.g., Cross et al. series of paraneoplastic optic neuritis patients; Chan's textbook on primary intraocular lymphoma) as well as publications published after 2000. References from select studies were additionally included. Given the sparsity of literature on many subjects, most publications were included during this time frame in our review. There are several ophthalmic entities that the oncologist should be aware of including leukemic optic nerve infiltration, PVRL, paraneoplastic syndromes as they related to the eye, and adverse effects of therapeutics. Unfortunately, given the rarity of some of these entities [e.g., paraneoplastic optic neuropathy (PON), cancer-associated retinopathy (CAR)], diagnosis can be difficult and treatment options are often limited. Oncologists can develop a set of basic ophthalmology examination skills that will help to triage and manage patient eye complaints. In certain instances, oncologists have the potential to avert devastating vision loss with early recognition of neuro-ophthalmic complications.

Autoimmune basal ganglia encephalitis associated with anti-recoverin antibodies: A case report

Autoimmune basal ganglia encephalitis causes neurological symptoms such as parkinsonism associated with basal ganglia lesions. Here, we report a case of autoimmune basal ganglia encephalitis without retinal lesions or malignancy harboring anti-recoverin antibodies. The patient was a 67-year-old Japanese woman who developed anorexia, parkinsonism, and disturbance of consciousness 7 days before admission. Brain magnetic resonance imaging showed hyperintense bilateral basal ganglia lesions on fluid-attenuated inversion recovery images. 18F-fluorodeoxyglucose-positron emission tomography showed no malignancy in the trunk, and dopamine transporter single-photon emission computed tomography with dopamine transporters revealed reduced radiotracer uptake in the basal ganglia. Further, anti-recoverin IgG antibodies were detected in serum immunoblot. Based on the clinical and imaging findings, the patient was diagnosed with autoimmune basal ganglia encephalitis with anti-recoverin antibodies and administered high-dose immunoglobulins (HD-IVIG), which led to an improvement in clinical symptoms. Anti-recoverin antibodies are paraneoplastic antibodies that explicitly bind to Ca2+-binding proteins in the retina and cause retinopathy. This pathological sequence is defined as cancer-associated retinopathy (CAR). However, in our case, autoimmune basal ganglia encephalitis developed without CAR syndrome or malignancy. Clinicians should be aware of the possibility of autoimmune basal ganglia encephalitis showing anti-recoverin antibodies but no CAR syndrome or malignancy, which should be treated with HD-IVIG therapy.

Off-Label Use of 0.19 mg Fluocinolone Acetonide Intravitreal Implant: A Systematic Review.

Corticosteroids are used in a variety of ophthalmological diseases. One challenge faced by ophthalmologists is to deliver corticosteroids to the posterior segment of the eye with efficacy and safety. Sustained-release corticosteroid implants may be the answer to this problem. The 0.19 mg fluocinolone acetonide (FAc) implant (Iluvien®) releases FAc for 36 months, and it is approved for the treatment of diabetic macular edema (DME) and noninfectious uveitis. We decided to do a systematic review to acknowledge in which other diseases FAc implant is being used off-label. A literature search was performed in the following three electronic databases: PubMed, Scopus, and Web of Science (from January 1st, 2000, to September 20th, 2020), using the following query: (“Fluocinolone Acetonide” OR Iluvien®) AND (“eye” OR “ocular” OR “intravitreal).” A total of 11 papers were included, and the use of FAc implant was analyzed in the following diseases: radiation-induced maculopathy (RM); paraneoplastic visual syndromes (melanoma-associated retinopathy (MAR) and cancer-associated retinopathy (CAR)); Sjogren's syndrome-related keratopathy; retinal vein occlusion (RVO); cystoid macular edema (CME); diabetic retinal neurodegeneration (DRN); and retinitis pigmentosa (RP). FAc implant may be a potential treatment for these diseases; however, the level of scientific evidence of the included studies in this review is limited. Further studies with larger cohorts and longer follow-ups are needed to validate this data.

Presumed cancer-associated retinopathy (CAR) mimicking Sudden Acquired Retinal Degeneration Syndrome (SARDS) in canines.

ObjectiveTo describe functional and structural features of presumed cancer‐associated retinopathy (CAR) mimicking sudden acquired retinal degeneration syndrome (SARDS) in dogs and describe treatment outcomes.AnimalsSubjects were 17 dogs from 8 eight US states and Canada diagnosed with SARDS or immune‐mediated retinitis (IMR) by 12 ophthalmologists. Nine eyes from seven deceased patients were used for microarray (MA), histology, or immunohistochemical (IHC) analysis.ProceduresDogs underwent complete ophthalmic examination, including retinal photography, optical coherence tomography (OCT), chromatic pupil light reflex testing (cPLR), and electroretinography (ERG), in addition to complete systemic examination. Histology, microarray, and IHC analysis were performed in CAR retinas to evaluate histological and molecular changes in retinal tissue.ResultsNone of the patients evaluated satisfied previously established criteria for diagnosis of SARDS (flat ERG+ no red – good blue PLR), and all were diagnosed with IMR. All patients were diagnosed with a cancer: meningioma (24%), sarcoma (18%), pituitary tumor (12%), and squamous cell carcinoma (12%), other (34%). Median survival time was 6 months from diagnosis (range 1‐36 months). Most frequent systemic abnormalities were as follows: proteinuria (78%); elevated liver enzymes (47%); and metabolic changes (PU/PD, polyphagia – 24%). Immunosuppressive therapy resulted in the reversal of blindness in 44% of treated patients, with 61% of all treated patients recovering and/or maintaining vision. Median time for preservation of vision was 5 months (range 1‐35 months).ConclusionsObserved changes are highly suggestive of immune‐mediated damage in IMR‐CAR eyes. A relatively high percentage of patients with CAR responded positively to immunosuppressive therapy.

Ocular Paraneoplastic Syndromes.

Ocular-involving paraneoplastic syndromes present a wide variety of clinical symptoms. Understanding the background pathophysiological and immunopathological factors can help make a more refined differential diagnosis consistent with the signs and symptoms presented by patients. There are two main pathophysiology arms: (1) autoimmune pathomechanism, which is presented with cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), cancer-associated cone dysfunction (CACD), paraneoplastic vitelliform maculopathy (PVM), and paraneoplastic optic neuritis (PON), and (2) ectopic peptides, which is often caused by tumor-expressed growth factors (T-exGF) and presented with bilateral diffuse uveal melanocytic proliferation (BDUMP). Meticulous systematic analysis of patient symptoms is a critical diagnostic step, complemented by multimodal imaging, which includes fundus photography, optical coherent tomography, fundus autofluorescence, fundus fluorescein angiography, electrophysiological examination, and sometimes fundus indocyjanin green angiography if prescribed by the clinician. Assessment of the presence of circulating antibodies is required for diagnosis. Antiretinal autoantibodies are highly associated with visual paraneoplastic syndromes and may guide diagnosis by classifying clinical manifestations in addition to monitoring treatment.

Cancer-Associated Retinopathy in Patients with Newly Diagnosed Breast Tumor.

Introduction:Paraneoplastic neurological syndromes (PNS) are rare neurological conditions and they are mostly triggered by autoimmune mechanisms. Cancer-related retinopathies (CAR) are even rarer and commonly related with breast tumor in woman. This limits our knowledge about pathophysiology of CAR. In this study, we question the association between histopathological findings and onconeural antibodies in breast cancer.Method:Thirty-two patients with newly diagnosed breast cancer admitted to the oncology outpatient clinic were included in the study. None of the participants have visual complaints. After the neurological examination of the patients, two tubes of 5 cc venous blood were obtained by screening onconeuronal antibodies. Samples were investigated in ASDETAE (İstanbul University Experimental Medicine Research Institute).Results:Patients included in the study included one patinet (3.1%) with grade 1, 14 patients (43.8%) with grade 2 and 17 patients (53.1%) with grade 3 invasive breast cancer. Perineural invasion was detected in 5 (15.6%) patients. Progesterone receptor positivity was found in 26 (81.2%) patients and estrogen receptor positivity was found in 27 (84.4%) patients. In 7 (21.9%) patients, CERBB2 was positive and in 25 (78.1%) patients, Ki 67 was positive. A total of 12 (37.5%) patients had onconeuroneal antibody positivity. Antibody positivity was significantly higher in patients with high grade tumor (p=0.008).Conclusion:There may be a relationship between tumor grade and the presence of onconeuronal antibodies in breast cancer patients. By the detection of new biochemical markers, significant contribution can be made to the early diagnosis and treatment of underlying cancer.

A Case of Maxillary Sinus Small Cell Carcinoma with Cancer-associated Retinopathy

A Case of Maxillary Sinus Small Cell Carcinoma with Cancer-associated Retinopathy

Pathophysiology of Cancer-associated Retinopathy

Pathophysiology of Cancer-associated Retinopathy

Masqueraders of multiple evanescent white dot syndrome (MEWDS).

To describe disorders that can masquerade as multiple evanescent white dot syndrome (MEWDS). Retrospective, multicenter case series. Patients who presented with clinical findings compatible with a diagnosis of MEWDS but were ultimately diagnosed with an alternative inflammatory, infectious, or neoplastic disorder. Clinical records and multimodal imaging findings including fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and OCT angiography (OCTA) were analyzed. Inclusion criteria to be defined as a masquerade syndrome for MEWDS included the presence of disseminated grayish-white outer retinal spots that were hyperautofluorescent on FAF and associated with ellipsoid zone (EZ) disruption on OCT. Twenty-two eyes of 13 patients were identified. All patients presented with the classic findings of MEWDS listed above. A MEWDS-like presentation was bilateral in nine of 13 patients (69%). Final diagnosis was determined on the basis of additional investigations including serologies and biopsy. These diagnoses included syphilis (three patients), lymphoma (three patients), idiopathic multifocal choroiditis (two patients), idiopathic retinal phlebitis (one patient), idiopathic acute zonal occult outer retinopathy (one patient), sarcoidosis (one patient), tuberculosis (one patient), and cancer-associated retinopathy (one patient). The outer retinal lesions and imaging findings resolved with treatment for the associated systemic disorders. Widespread grayish-white outer retinal spots associated with hyperautofluorescence on FAF and disruption of the EZ on OCT are not pathognomonic for MEWDS. A high index of suspicion must be maintained for masqueraders of MEWDS, which can include serious inflammatory, infectious, and neoplastic disorders.

Paraneoplastic causes of vision loss

AbstractParaneoplastic occular disorders are clinical syndromes resulting from nonmetastatic systemic effects of underlying cancers. While not fully elucidated the underlying mechanism is presumed to be a cross reaction of the immune reaction to the underlying cancer that attaches normal host tissue. This cross reaction can happen in a variety of occular tissues resulting in dysfunction of the retina, optic nerve and tear film and extraocular muscles. (1‐3). The lists of cancers and associated antibodies is constantly growing but, in many cases, the clinical phenotype is very similar. Cancer associated retinopathy (CAR) for example is associated with several antibodies including anti‐recoverin, anti‐alpha enolase, anti‐transducin, and anti‐carbonic anhydrase(2, 3). Each of these antibodies have been associated with a distinct cancer including skin, genital and small cell lung cancer (SCLC). CAR causes a slowly progressive asymmetric vision loss due to damage to retinal ganglion cells and bipolar cells which can result in optic nerve pallor. Melanoma associated retinopathy (MAR), similarly can present with nyctalopia, photopsia’s and loss of peripheral vision. The antibodies associated with MAR; anti‐transducin, anti‐rhodopsin and anti‐arrestin typically attack the on bipolar system. As with CAR the visual dysfunction can predate the discovery of the cancer. Conversely, autoimmune related retinopathy and optic neuropathy occurs without an underlying cancer and is associated with anti‐GAD and anti‐ anti‐collapsin‐responsive mediator protein‐5 (CRMP‐5) antibodies(2). In all three cases electroretinograms are key to making the diagnosis as the objective clinical examination can be normal. Electroretinograms are key to distinguishing retinal dysfunction which can cause an optic neuropathy from a paraneoplastic optic neuropathy (PON). The classical antibody in PON is. CRMP‐5 is associated with SCLC and thymoma and can produce optic disc edema with subsequent atrophy. The treatment and management of occular paraneoplastic disorders requires appropriate investigations to look for an underlying cancer. Knowing the specific antibody associated with the disorder can be helpful in this regard and in some cases a panel of antibody tests is appropriate in the right clinical context(4). Treatment of the underlying cancer can, in some cases, can lead to a resolution of the clinical symptoms. Symptom control can also be accomplished using immunosuppression with steroids or IVIG and in some cases plasmapheresis has been useful(4, 5).References Grewal DS, Fishman GA, Jampol LM. Autoimmune retinopathy and antiretinal antibodies: a review. Retina. 2014;34(5):827–45. Bataller L, Dalmau J. Neuro‐ophthalmology and paraneoplastic syndromes. Current opinion in neurology. 2004;17(1):3–8. Rahimy E, Sarraf D. Paraneoplastic and non‐paraneoplastic retinopathy and optic neuropathy: evaluation and management. Survey of ophthalmology. 2013;58(5):430–58. Pelosof LC, Gerber DE. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc. 2010;85(9):838–54. Damek DM. Paraneoplastic Retinopathy/Optic Neuropathy. Current treatment options in neurology. 2005;7(1):57–67.

Occurrence of major anti-retinal autoantibodies associated with paraneoplastic autoimmune retinopathy

Autoantibodies (AAbs) against retinal antigens can be found in patients with cancer and unexplained vision loss unrelated to the cancer metastasis. Cancer-associated retinopathy (CAR) is a rare paraneoplastic visual syndrome mediated by AAbs. Our goal was to determine whether CAR patients with different malignancies have a specific AAb or repertoire of AAbs that could serve as biomarkers for retinal disease. We found AAbs against 12 confirmed retinal antigens, with α-enolase being the most frequently recognized. The significant finding of the study was a high incidence of anti-aldolase AAbs in colon-CAR, anti-CAII in prostate-CAR, and anti-arrestin in skin melanoma patients thus these AAbs could serve as biomarkers in the context of clinical presentation and could support the diagnosis of CAR. However, a lack of AAb restriction to any one antigenic protein or to one retinal cellular location makes screening for a CAR biomarker challenging.