title: 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.
authors.affiliation: Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002, India. drnaina7@gmail.com
authors.forename: Naina R
authors.initials: NR
authors.lastname: Bamrolia
authors.forename: Ritu
authors.initials: R
authors.lastname: Arora
authors.forename: Usha
authors.initials: U
authors.lastname: Yadava
mesh_terms: D000328:Adult; D003228:Conjunctiva / Q000473:pathology*; D003231:Conjunctivitis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007634:Keratitis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D009336:Necrosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D015423:Scleritis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents