Types
The disease may be subdivided into paraneoplastic and non-paraneoplastic AIR. When related to melanoma, it is referred to as MAR, and when related to other cancers, it is called CAR.
Cancer-associated retinopathy
A division of AIR, cancer-associated retinopathy is a paraneoplastic syndrome , which is a disorder caused by an immune system response to an abnormality. Autoimmune antibodies attack target proteins in retinal photoreceptor cells. The proteins targeted as antigenic are recovering, may α‐enolase, and transducin.[rx] This autoimmune response leads to photoreceptor cell death in ratina.[rx] It causes progressive vision loss that can lead to blindness.[rx] CAR is typically associated with the anti-recoverin antibody.[rx]
Melanoma-associated retinopathy
Retinal bipolar cells in the retina transmit signals to react with the antibodies, leading to cell death. Although it is less prevalent than CAR, diagnosed cases of MAR continue to increase while CAR numbers decrease.[rx]
Symptoms
Both CAR and MAR share the about to same symptoms. This is because they are both paraneoplastic syndromes. AIR symptoms are numerous and shared by many other diseases.[rx]
- Patients present with vision problem with bilateral and asymmetric scotomas, photopsia, visual field defects, with rapidly progressive visual loss in late-onset.
- In the initial stage, fundus examination is unremarkable, and in late stages, there is limited retinal vascular attenuation, with or without optic disc pallor, associated or not with intraocular inflammation and with no evidence of degenerative retinal disease.
- Painless Vision Loss
- Blind Spots in Vision
- Photopsia
- Nyctalopia
- Scotomas
- Dislike/avoidance of light
- Loss of contrast sensitivity
- Decreased night vision
- Subacute vision loss
- Eye floaters or flashes
- Blind spots
- Decreased color vision
- Bilateral, slowly progressive vision loss
- Prolonged glare after light exposure
- Decreased color perception
- Central blindspots
- Poor night vision
Diagnosis
Ophthalmologic and complementary examinations such as full-field electroretinogram, optical coherence tomography, visual field and fundus autofluorescence, help the diagnosis.[rx]
Diagnosis of AIR can be difficult due to indentify of symptoms with other disorders.[rx] Examination of the fundus of ratina (inner surface of eye) can show no results or it can show narrowing of the blood vessels, the abnormal color of the optic disc, and retinal atrophy with spasm.[rx][rx] Fundus examination results are not indicate of autoimmune retinopathy but they are used to initiate the diagnostic process. An electroretinogram (eye test used to see abnormalities in the retina) is used to detect AIR. An abnormal electroretinogram (ERG) with respect to light and dark adaptations indicates AIR.[rx] The ERG also allows differentiation between cancer-associated retinopathy and melanoma-associated retinopathy.[rx] If the ERG shows cone responses, CAR can be prematurely diagnosed.[rx] If the ERG shows a significant decrease in b-wave amplitude, MAR can be prematurely diagnosed.[rx][rx] To confirm, analysis for anti-retinal antibodies through Western blotting of serum collected from the patient is done.[rx][rx]
Treatment
Due to the difficulty of diagnosis, managing this disease is a challenge. For this reason, there is no established treatment for AIR. Clinicians try to reduce and control the autoimmune system attack to prevent any irreversible retinal damage.[rx] Methods of treatment include intravenous immunoglobulin (IVIG), plasmapheresis, and corticosteroids.[rx]
Immunoglobulin
Immunoglobulin samples are get from a large pool of healthy, matched donors (10000 – 20000).[rx] The immunoglobulin mixture is then administered through IV at a rate of 0.4g/kg/day for 5 days.[rx] Antibodies in the IVIG mixture interact with binding in the site of the disease-associated antibodies (such as anti-recoverin antibodies).[rx] This prevents binding to proteins targeted to disease and reduces disease activity.[rx] Responses to this treatment can vary and are effects if the patient is diagnosed with any type of cancer.[rx] Patients who respond positively improve in the clarity of their vision and their visual field.[rx]
Plasmapheresis
Plasmapheresis involves separating blood into two parts of blood cells and plasma.[11] The blood plasma components, such as the antibodies, are treated outside of the body. After removal of the disease-associated antibodies, the blood cells and plasma are transferred back into the body.[rx] Response to this treatment depends on how much and quantity retinal damage has been done. Patients who respond positively show significant visual gains.[rx]
Corticosteroids
Corticosteroids are administered through intravenous IV or orally. They cause lymphocytopenia, a condition where white blood cell levels are abnormally low.[rx] Corticosteroids cause white blood cell death, lowering their amount throughout the body.[rx] They also caused the white blood cells to recirculate away from the area of damage (the retina).[rx] This minimizes damage caused by the antibodies produced by the white blood cells. Often, this is treatment is combined with the plasmapheresis process.[rx] Instead of treating the plasma and blood cells, they are healthy donor mixture. Patients who respond positively show improved visual fields and almost complete disappearance of anti-retinal antibodies.[rx]
Dr. Md. Harun Ar Rashid, MPH, MD, PhD, is a highly respected medical specialist celebrated for his exceptional clinical expertise and unwavering commitment to patient care. With advanced qualifications including MPH, MD, and PhD, he integrates cutting-edge research with a compassionate approach to medicine, ensuring that every patient receives personalized and effective treatment. His extensive training and hands-on experience enable him to diagnose complex conditions accurately and develop innovative treatment strategies tailored to individual needs. In addition to his clinical practice, Dr. Harun Ar Rashid is dedicated to medical education and research, writing and inventory creative thinking, innovative idea, critical care managementing make in his community to outreach, often participating in initiatives that promote health awareness and advance medical knowledge. His career is a testament to the high standards represented by his credentials, and he continues to contribute significantly to his field, driving improvements in both patient outcomes and healthcare practices.
