Korean J Ophthalmol.  2011 Aug;25(4):275-277. 10.3341/kjo.2011.25.4.275.

A Case of Necrotizing Keratoscleritis in Primary Sjogren's Syndrome

• Choi W
• Lee SS
• Park YG
• Yoon KC

• KMID: 1018417
• DOI: http://doi.org/10.3341/kjo.2011.25.4.275

Abstract

We report on a case of necrotizing keratoscleritis in primary Sjogren's syndrome. A 66-year-old female patient who was complaining of ocular pain, tearing and decreased vision in her right eye for the previous two days was admitted to our hospital. Visual acuity in the right eye was hand movement, and initial examination showed a 3.0 x 1.8 mm uveal mass bulging through a corneoscleral melting site in the nasal region of the right eye. Positive anti-nuclear antibody was identified at a titer of 1:320 with a speckled pattern, and both Sjogren's syndrome A and Sjogren's syndrome B antibody tests were positive, with titers >200 U/mL. A technetium 99m pertechnetate salivary scan revealed chronic sialoadenitis in the submandibular glands. We diagnosed the lesion as necrotizing keratoscleritis due to primary Sjogren's syndrome. A corneoscleral patch graft was performed, followed by immunosuppression including oral cyclosporin and topical prednisolone. During a follow-up period of 12 months, the corneoscleral graft was well maintained with no recurrence.

Keyword

Immunosuppression; Necrotizing scleritis; Sjogren's syndrome

MeSH Terms

Aged
Diagnosis, Differential
Disease Progression
Female
Follow-Up Studies
Glucocorticoids/administration & dosage
Humans
Keratitis/drug therapy/*etiology/pathology
Necrosis
Ophthalmic Solutions
Scleritis/drug therapy/*etiology/pathology
Sjogren's Syndrome/*complications/drug therapy/pathology
Visual Acuity

Figure

• 

  Fig. 1 Slit lamp photographs. (A) A 3 × 1.8 mm mass of uveal tissue was observed through a corneoscleral melting site. (B) Twelve months later, the corneoscleral patch graft was maintained without inflammation.

Reference

1. Mavragani CP, Moutsopoulos HM. Conventional therapy of Sjogren's syndrome. Clin Rev Allergy Immunol. 2007. 32:284–291.

2. Cohen KL. Sterile corneal perforation after cataract surgery in Sjogren's syndrome. Br J Ophthalmol. 1982. 66:179–182.

3. Vivino FB, Minerva P, Huang CH, Orlin SE. Corneal melt as the initial presentation of primary Sjogren's syndrome. J Rheumatol. 2001. 28:379–382.

4. Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease. Nat Clin Pract Rheumatol. 2007. 3:219–226.

5. Ahmadi-Simab K, Lamprecht P, Nolle B, et al. Successful treatment of refractory anterior scleritis in primary Sjogren's syndrome with rituximab. Ann Rheum Dis. 2005. 64:1087–1088.