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ACR Open Rheumatol. 2019 Jul;1(5):292-302. doi: 10.1002/acr2.1033. Epub 2019 Jun 7.

Reproducibility of Ocular Surface Staining in the Assessment of Sjögren Syndrome-Related Keratoconjunctivitis Sicca: Implications on Disease Classification.

Author information

Astrid Rasmussen, MD, PhD: Oklahoma Medical Research Foundation, Oklahoma City, and Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, México.
Donald U. Stone, MD: Johns Hopkins University, Baltimore, Maryland (current address: Dean McGee Eye Institute, University of Oklahoma Health Sciences Center, Oklahoma City).
C. Erick Kaufman, MD, Lida Radfar, DDS, MS, David M. Lewis, DDS: University of Oklahoma, Oklahoma City.
Kimberly S. Hefner, DO: Hefner Eye Care and Optical Center, Oklahoma City, Oklahoma.
Nicole R. Fram, MD: David Geffen School of Medicine, University of California, Los Angeles.
Rhea L. Siatkowski, MD: University of Oklahoma and Dean McGee Eye Institute, Oklahoma City.
Andrew J. W. Huang, MD: School of Medicine, Washington University in St. Louis, St. Louis, Missouri (current address: University of Minnesota).
James Chodosh, MD, MPH: Massachusetts Eye and Ear and Harvard University, Boston (current address: Dean McGee Eye Institute, University of Oklahoma Health Sciences Center, Oklahoma City).
Pablo T. Rasmussen: Harvard University, Cambridge, Massachusetts.
Dustin A. Fife, PhD (current address: Oklahoma Medical Research Foundation, Oklahoma City), Nathan Pezant, MS, Kiely Grundahl, BS, Courtney G. Montgomery, PhD: Oklahoma Medical Research Foundation, Oklahoma City.
Michael H. Weisman, MD, Swamy Venuturupalli, MD, Daniel J. Wallace, MD: Cedars-Sinai Medical Center, Los Angeles, California.
Nelson L. Rhodus, DMD, MPH: School of Dentistry, University of Minnesota, Minneapolis.
Michael T. Brennan, DDS, MHS: Carolinas Medical Center, Charlotte, North Carolina.
Christopher J. Lessard, PhD, Kathy L. Sivils, PhD: Oklahoma Medical Research Foundation and University of Oklahoma, Oklahoma City.
R. Hal Scofield, MD: Oklahoma Medical Research Foundation, University of Oklahoma, and Department of Veterans Affairs Medical Center, Oklahoma City.



The objective of this study was to assess the performance and reproducibility of the two currently used ocular surface staining scores in the assessment of keratoconjunctivitis sicca in Sjögren syndrome (SS) research classification.


In a multidisciplinary clinic for the evaluation of sicca, we performed all tests for the American European Consensus Group (AECG) and the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria, including the van Bijsterveld score (vBS) and the Ocular Staining Score (OSS), in 994 participants with SS or with non-SS sicca. We analyzed the concordance between the scores, the diagnostic accuracy and correlation with clinical variables, and interrater and intrasubject reproducibility.


A total of 308 (31.1%) participants had a discordant vBS and OSS that was due to extra corneal staining points in the OSS. The presence of one or more of the additional points was highly predictive of SS classification (odds ratio = 3.66; P = 1.65 × 10e-20) and was associated with abnormal results of all measures of autoimmunity and glandular dysfunction. Receiver operating characteristic curves showed optimal cutoff values of four for the vBS (sensitivity = 0.62; specificity = 0.71; Youden's J = 0.33) and five for the OSS (sensitivity = 0.56; specificity = 0.75; Youden's J = 0.31). Notably, there was very poor consistency in interobserver mean scores and distributions (P < 0.0001) and in intrasubject scores after a median of 5.5 years (35% changed status of the ocular criterion).


Ocular surface staining scores are useful for SS research classification; however, they are subject to significant interrater and intrasubject variability, which could result in changes in classification in 5%-10% of all subjects. These results highlight the need for objective and reproducible markers of disease that have thus far remained elusive for SS.

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