Characteristics of Korean primary Sjogren's syndrome and comparison of 3 different classification criteria for primary Sjogren's syndrome: data from Korean KISS Cohort

Abstract

Background/Purpose: The objective of this study is to introduce the clinical and laboratory characteristics of Korean primary Sjogren’s syndrome (pSS) patients enrolled in Korean Initiative of Sjogren’s Syndrome (KISS). We also sought to compare the performance of newly proposed 2016 American College of Rheumatology (ACR)/European League-Against Rheumatism (EULAR) criteria to 2002 American-European Consensus Group (AECG) and 2012 ACR classification criteria for primary pSS in a well characterized registry.

Methods: Patients with pSS from 12 university affiliated hospitals in Korea were enrolled from Oct 2013 to JAN 2017. The patients were diagnosed with pSS by either fulfilling 2002 AECG or 2012 ACR classification criteria. Data of clinical manifestations and various laboratory findings were obtained.

Results: Data at inclusion were available in 458 patients. The mean age was 51.9 ± 11.8 years. Four hundred fifty patients (98.3%) were female. Mean disease duration was 33.9 ± 46.7 months. Most common extraglandular manifestation was arthralgia (47.8%) followed by Raynaud’s phenomenon (15.7%) and lymphadenopathy (13.5%). Median [interquartile range] ESSDAI and ESSPRI was 2 [0-5], 5 [4-6.7], respectively. Among 458 patients, 328 patients had sufficient data to determine the fulfillment of each criteria. All three criteria were met by 307 patients. Among 3 patients by whom 2016 ACR/EULAR criteria were not met, one 2002 AECG- 2012 ACR+ had negative anti Ro or La while positive antinuclear antibody and rheumatoid factor with more than 3 ocular staining score. Two 2002 AECG+ 2012 ACR- showed no ocular sign and negative anti Ro or La while had positive focus score. Ninety-six patients had results of all items in 2016 criteria, 95 met the criteria.

Conclusion: We successfully established a nationwide pSS registry in Korea, which represents the characteristics of Korean pSS patients. The newly proposed 2016 ACR/EULAR criteria were met by most of the patients diagnosed with pSS according to previous criteria.