Unusual presentation of a case of Sjogren's syndrome with neurological and ocular manifestation

Abstract Sjogren's syndrome is an autoimmune disease that commonly presents to the ophthalmologist as a dry eye disease. We report an unusual presentation of a case of Sjogren's syndrome. A 33-year-old man presented with lid swelling, pseudomembraneous conjunctivitis with central corneal epithelial defect and history of limb weakness for past 2 years. There was progressive enlargement of the epithelial defect and conjunctival-scleral necrosis developed during follow-up. Evaluation for underlying connective tissue disorder was positive for SS-B/La antibody and, the rheumatoid factor, anti nuclear antibody, anti neutrophil cytoplasmic antibody titres were negative. Patient was diagnosed as a case of Primary Sjogren's syndrome. Resolution of the conjunctival-scleral necrosis occurred following treatment with combination of cyclosporine and prednisolone. Paramedian tarsorrhaphy was performed to promote healing of the corneal epithelium. The corneal epithelium healed, conjunctival-scleral necrosis resolved completely and the visual acuity improved to 6/36 in the right eye after 3 months of immunosuppressive therapy. The immunosuppressive therapy was discontinued after completion of 6 months of treatment. After 6 months follow-up of stopping immunosuppressive therapy, the ocular and systemic condition is stable.