The National Institutes of Health-funded Sjögren's Syndrome International Registry (called Sjögren's International Collaborative Clinical Alliance [SICCA]) is developing standardized classification criteria for Sjögren syndrome (SS) and is creating a biospecimen bank for future research. Eight SICCA ophthalmologists developed a new quantitative ocular grading system (SICCA ocular staining score [OSS]), and we analyzed OSS distribution among the SICCA cohort and its association with other phenotypic characteristics of SS. The SICCA cohort includes participants ranging from possibly early SS to advanced disease. Procedures include sequenced unanesthetized Schirmer test, tear break-up time, ocular surface staining, and external eye examination at the slit lamp. Using statistical analyses and proportional Venn diagrams, we examined interrelationships between abnormal OSS (≥3) and other characteristics of SS (labial salivary gland [LSG] biopsy with focal lymphocytic sialadenitis and focus score >1 positive anti-SS A antibodies, anti-SS B antibodies, or both).
Results
Among 1208 participants, we found strong associations between abnormal OSS, positive serologic results, and positive LSG focus scores (P < .0001). Analysis of the overlapping relationships of these 3 measures defined a large group of participants who had KCS without other components of SS, representing a clinical entity distinct from the KCS associated with SS.
Conclusions
This new method for assessing KCS will become the means for diagnosing the ocular component of SS in future classification criteria. We find 2 forms of KCS whose causes may differ.
aDepartment of Ophthalmology, University of California, San Francisco, San Francisco, California
bDepartment of Orofacial Sciences, University of California, San Francisco, San Francisco, California
cDepartment of Epidemiology & Biostatistics, University of California, San Francisco, San Francisco, California
dDepartment of Ophthalmology, German Hospital and University of Buenos Aires, Buenos Aires, Argentina
eDepartment of Ophthalmology, Kanazawa Medical University, Ishikawa, Japan
fDepartment of Ophthalmology, Peking Union Medical College Hospital, Beijing, China
gDepartment of Ophthalmology, Copenhagen University Hospital, Glostrup, Denmark
hDepartment of Ophthalmology, King's College London, London, United Kingdom
Inquiries to Troy E. Daniels, 513 Parnassus Avenue, Box 0422, University of California, San Francisco, San Francisco, CA 94143–0422
ABSTRACT