BACKGROUND: The aim of this study was to evaluate the prevalence of long-term recurrence after topical nonpreserved methylprednisolone pulse therapy for the treatment of keratoconjunctivitis sicca (KCS) with Sjögren's syndrome. METHODS: A total of 106 eyes of 53 Sjögren's syndrome patients were included in the study. All patients were treated with topical nonpreserved 1% methylprednisolone solution. Initial therapy consisted of eyedrops 4 times a day for 2 weeks, and then patients were reevaluated and tapered off the medication every 2 weeks until discontinuation. Tear film breakup time (TBUT), Schirmer test, corneal fluorescein staining, and subjective symptom scores were measured. Additionally, impression cytology of the bulbar conjunctiva was performed. The over-all success rates and success period were determined using the Kaplan-Meier survival curve. RESULTS: A reduction in subjective symptoms and fluorescein staining, and an improvement in TBUT and Schirmer test results, was observed after treatment (P<0.001). Impression cytology specimens revealed a significantly increased number of periodic acid-Schiff-positive cells after treatment. After the first pulse therapy, mean survival was 56.6 weeks and 11 (20.8%) patients recurred. After the second pulse therapy, mean survival was 72.4 weeks and only 1 patient recurred. No serious complications, including intraocular pressure elevation and cataract formation, were encountered during the entire follow-up period. CONCLUSIONS: Topical nonpreserved methylprednisolone pulse therapy proved to be a safe, effective long-term treatment of improving subjective and objective dry eye factors in KCS patients with Sjögren's syndrome.