Non-paraneoplastic autoimmune retinopathy that developed in fellow eye 10 years after onset in first eye: a case report

• Published in: BMC ophthalmology 2020-04, Vol.20 (1), p.132-132, Article 132
• Main Authors: Miura, Gen, Baba, Takayuki, Iwase, Takehito, Ohde, Hisao, Kanda, Atsuhiro, Saito, Wataru, Ishida, Susumu, Yamamoto, Shuichi
• Format: Article
• Language: English
• Subjects: Air bases; Alpha-enolase; Analysis; Antibodies; Autoantibodies - blood; Autoimmune diseases; Autoimmune Diseases - diagnosis; Autoimmune Diseases - etiology; Autoimmune Diseases - therapy; Autoimmune retinopathy; Blindness; Blindness - etiology; Case Report; Case reports; Defects; Edema; Electroretinography; Endoscopy; Eye; Fluorescein Angiography; Glucocorticoids - therapeutic use; Health aspects; Humans; Immunoblotting; Immunoglobulins; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Inflammation; Male; Medical research; Middle Aged; Non paraneoplastic autoimmune retinopathy; Paraneoplastic Syndromes, Ocular - diagnosis; Paraneoplastic Syndromes, Ocular - etiology; Paraneoplastic Syndromes, Ocular - therapy; Patients; Phosphopyruvate hydratase; Phosphopyruvate Hydratase - immunology; Plasma Exchange; Retina; Retinal Diseases - diagnosis; Retinal Diseases - etiology; Retinal Diseases - therapy; Retinopathy; Steroid hormones; Time Factors; Tomography, Optical Coherence; Visual Acuity; Visual field; Visual Field Tests
• ISSN: 1471-2415; 1471-2415
• DOI: 10.1186/s12886-020-01414-z

Evidence-based criteria for the treatment of autoimmune retinopathy (AIR) have not been established. The pathology and clinical features of each antibody causing AIR, and its long-term course are still undetermined. We report our findings in a case of non-paraneoplastic AIR (npAIR) that developed in the fellow eye 10 years after the onset in the first eye. Our patient had photophobia in both eyes and a rapidly progressing visual field defect in his right eye at the initial examination. He was diagnosed with non-paraneoplastic AIR based on the clinical findings and immunoblot analyses for anti-retinal antibodies, and he was treated with steroids. Ten years later, a visual field defect developed in the fellow eye, and a diagnosis of npAIR was made. Immunoblot analyses were positive for anti-α-enolase antibodies. He was treated with steroids, immunosuppressants, and plasma exchange. However, the response to the treatment was poor and both eyes eventually became blind. As best we know, this is the first case report of npAIR that developed in the fellow eye over 10 years after the development in the first eye. Long-term follow-up and a search for tumor lesions are necessary in cases of npAIR. Further understanding of the long-term course of AIR can contribute to an understanding of the pathology and treatment of npAIR.