Paraneoplastic Retinopathy: A Novel Autoantibody Reaction Associated with Small-cell Lung Carcinoma

• Published in: Journal of neuro-ophthalmology Vol. 17; no. 2; pp. 77 - 83
• Main Authors: Murphy, Marjorie A, Thirkill, Charles E, Hart, William M
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Williams & Wilkins 01.06.1997; Lippincott Williams & Wilkins
• Subjects: Aged; Antibodies, Neoplasm - immunology; Autoantibodies - analysis; Biological and medical sciences; Blotting, Western; Carcinoma, Small Cell - immunology; Carcinoma, Small Cell - pathology; Carcinoma, Small Cell - therapy; Eye Proteins - immunology; Glucocorticoids - therapeutic use; Humans; Lung Neoplasms - immunology; Lung Neoplasms - pathology; Lung Neoplasms - therapy; Male; Medical sciences; Ophthalmology; Paraneoplastic Syndromes - immunology; Paraneoplastic Syndromes - pathology; Paraneoplastic Syndromes - therapy; Plasmapheresis; Prednisone - therapeutic use; Retina - immunology; Retinal Diseases - immunology; Retinal Diseases - pathology; Retinal Diseases - therapy; Retinopathies; Visual Acuity; Visual Fields; Human; Lung disease; Retinopathy; Antibody; Respiratory disease; Autoantibody; Malignant tumor; Bronchopulmonary; Case study; Eye disease; Neuron; Immunological investigation; Bronchus disease; Paraneoplastic syndrome; Small cell carcinoma
• ISSN: 1070-8022; 1536-5166
• DOI: 10.1097/00041327-199706000-00001

We present the case of a 74-year-old man with rapidly progressive bilateral visual loss, optic disc pallor, retinal arteriolar attenuation, and an abnormal electroretinogram with a 90% reduction in cone function and a 50% reduction in rod function. He was examined for a suspected cancer-associated retinopathy (CAR). Although he was found not to have expressed the previously reported 23-kd CAR antibody, high titers were found of an antibody to a 60-kd retinal protein, which as yet remains unidentified. An initial clinical search for an underlying cancer was unsuccessful, but 2 months later a mediastinal mass was found on chest x-rays, and biopsy confirmed a diagnosis of small-cell lung carcinoma. Combined therapy with oral corticosteroids and plasmapheresis resulted in a recovery of vision from counting fingers to 20/200 in the right eye and 20/40 to 20/25 in the left eye. Conventional chemotherapeutic management of the small-cell lung carcinoma was instituted, and the modest visual recovery was maintained. The visual improvement as well as lung tumor regression were accompained by a decline in antibody titers from 1:2,000 pretreatment to 1:200 during the course of therapy. The absence of reactivity with the previously described 23-kd retinal antigen of the CAR syndrome does not exclude the diagnosis of paraneoplastic retinopathy in patients fitting the clinical profile of this disease.