Comparison of the American-European Consensus Group Sjögren's syndrome classification criteria to newly proposed American College of Rheumatology criteria in a large, carefully characterised sicca cohort

Abstract

Objective To compare the performance of the American-European Consensus Group (AECG) and the newly proposed American College of Rheumatology (ACR) classification criteria for Sjögren's syndrome in a well-characterized sicca cohort, given ongoing efforts to resolve discrepancies and weaknesses in the systems. Methods In a multidisciplinary clinic for the evaluation of sicca, we assessed features of salivary and lacrimal gland dysfunction and autoimmunity as defined by tests of both AECG and ACR criteria in 646 participants. Global gene expression profiles were compared in a subset of 180 participants. Results Application of the AECG and ACR criteria resulted in classification of 279 and 268 participants with SS, respectively. Both criteria were met by 244 participants (81%). In 26 of the 35 AECG+/ACR- participants, the minor salivary gland biopsy focal score was ≥1 (74%), while 9 had positive anti-Ro/La (26%). There were 24 AECG-/ACR+ who met ACR criteria mainly due to differences in the scoring of corneal staining. All patients with SS, regardless of classification, had similar gene expression profiles, which were distinct from the healthy controls. Conclusion The two sets of classification criteria yield concordant results in the majority of cases and gene expression profiling suggests that patients meeting either set of criteria are more similar to other SS participants than to healthy controls. Thus, there is no clear evidence for increased value of the new ACR criteria over the old AECG criteria from the clinical or biological perspective. It is our contention, supported by this report, that improvements in diagnostic acumen will require a more fundamental understanding of the pathogenic mechanisms than is at present available.

The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren’s Syndrome Diagnosis

“…These criteria are to be applied to patients with signs/symptoms suggestive of SS, with diagnosis being determined by meeting at least 2 of the following three standardized, objective measures: traditional biomarker positivity (anti-SS-A/Ro and/or anti-SS-B/La) or positive rheumatoid factor (RF) and antinuclear antigen (ANA) titer C1:320; relevant labial salivary gland biopsy findings; and/or presence of keratoconjunctivitis sicca (KCS) ( Table 2). In a comparison of these two sets of guidelines in 646 individuals with sicca symptoms, a total of 303 participants were classified as having SS by either AECG criteria (n = 279) or 2012 ACR criteria (n = 268); 244 of the 303 (81%) diagnosed cases fulfilled both the AECG and ACR criteria, implying good, but not complete, concordant diagnostic results [37].…”

Ocular Manifestations of Sjögren Syndrome

“…1. SICCA Ocular staining score [22,25]. Total scores of 0 to 12 per eye assess the range of severity for dry eye.…”

“…Comparison of the Revised AECG and the ACR Classification criteria for SS are summarized in Table 1 [25]. In patients without any potentially associated disease, primary SS may be defined as follows: A.…”

Pathobiology of Sjögren Syndrome: What do we Know?

“…The diagnosis of SS has been the focus of extensive research, with two major classification strategies emerging in the last 12 years [11]. With the establishment of these consensus statements, there appears to be more consistency going forward regarding the diagnosis of this disorder [9,11,12].…”

“…With the establishment of these consensus statements, there appears to be more consistency going forward regarding the diagnosis of this disorder [9,11,12].…”