American College of Rheumatology classification criteria for Sjögren's syndrome: A data‐driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance Cohort

Abstract

Objectives We propose new classification criteria for Sjögren’s Syndrome (SS), which are needed considering the emergence of biological agents as potential treatments and their associated co-morbidity. These criteria target individuals with signs/symptoms suggestive of SS. Methods Criteria are based on expert opinion elicited using the Nominal Group Technique, and analyses of data from the Sjögren’s International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American-European-Consensus-Group (AECG) criteria, a model-based “gold standard” obtained from Latent Class Analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development Results Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 out of the following 3: Positive serum anti-SSA and/or anti-SSB or [positive rheumatoid factor and ANA ≥ 1:320]; Ocular staining score ≥ 3; Presence of focal lymphocytic sialadenitis with focus score ≥ 1 focus/4mm2 in labial salivary gland biopsies. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications. Conclusion These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.

Is minor salivary gland biopsy more than a diagnostic tool in primary Sjo¨gren׳s syndrome? Association between clinical, histopathological, and molecular features: A retrospective study

“…Different histological scores have been employed to describe glandular involvement during pSS [6][7][8][9][10], and as far as MSGs histology is concerned, its diagnostic role is widely accepted and currently considered the "gold standard." Indeed, MSG biopsy is considered 1 of the 4 objective criteria of American-European Consensus Group's classification and 1 of the 3 objective criteria of American College of Rheumatology's classification for pSS [11,12].…”

Effects of periodontal treatment on primary sjȫgren’s syndrome symptoms

“…The patients recruited comprised 14 of the 60 female pSS patients diagnosed according to the classification criteria of the American-European Consensus Group of 2002 14 and the criteria of the American Academy of Rheumatology of 2012, 15 aged between 35 and 70 years, and followed up at the Sjögren's Syndrome Service of the Hospital Clinics of the School of Medicine, University of Sao Paulo. The exclusion criteria were as follows: presence of active severe systemic involvement (neurological, pulmonary, renal or vasculitis involvement), smoking, current use of antibiotics or of phenytoin or calcium channel blockers, positive serology for HIV or hepatitis B or C, other autoimmune diseases (such as systemic lupus erythematosus, rheumatoid arthritis or systemic sclerosis), pregnancy or lactation, current chemotherapy and history of periodontal treatment, in the six months preceding the start of the study.…”

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

“…Diagnostic threshold was achieved by the presence of at least four of the six items, as long as one of the items was either positive biopsy histopathology or biomarker positivity. In 2012, a new set of criteria based on the National Institutes of Health--funded Sjögren's International Collaborative Clinical Alliance (SICCA) registry were published upon receiving provisional approval by the American College of Rheumatology (ACR) [36]. 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).…”