Vogt-Koyanagi-Harada syndrome and keratoconjunctivitis sicca

PURPOSE: To discover a relationship between Vogt-Koyanagi-Harada (VKH) syndrome and Sj?gren syndrome. DESIGN: Observational case-control study. METHODS: Sixteen Italian patients with VKH syndrome and 16 controls with diffuse uveitis underwent tear analysis and rheumatologic evaluation. Biopsy specimens of the minor salivary glands were obtained or salivary gland scintigraphy was performed in patients with xerostomia. RESULTS: The Schirmer test (P <.0001), break-up time (P <.0001), the Ferning test (P <.001), and fluorescein and rose bengal staining (P <.0001) resulted in changes in VKH. Biopsy was positive in two patients and scintigraphy in four. Human leukocyte antigen DR4 was found in 68.75% of VKH cases and in 83.3% of the cases with Schirmer test less than 3 mm. CONCLUSIONS: The incidence of keratoconjunctivitis sicca was higher in VKH syndrome patients than in controls. Two patients satisfied the criteria for Sj?gren syndrome, and two others had scintigraphy indicative of salivary gland involvement. The association of these two autoimmune disorders is suggested by the low incidence of VKH syndrome in Italy and might be related to HLA DR4.