No 5, 2012

Publication of the Month


May 05/12: 

ACR classification criteria for Sjögrens’ syndrome


Key message:

Case definition requires at least 2 of the following 3:
1)    positive serology for anti-SSA/Ro and/or anti-SSB/La (or positive RF and ANA titer ≥1:320),
2)    ocular staining score >3, or
3)    presence of focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm² in labial salivary gland biopsy samples


Shiboski SC, Shiboski CH, Criswell LA, et al., Sjögren's International Collaborative Clinical Alliance (SICCA) Research Groups
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
Arthritis Care & Research 2012;64:475-487


Since 1965 11 classification or diagnostic criteria for Sjögren’s syndrome (SS) have been published. The emergence of biologic agents as potential treatments combined with the use of old criteria lead to a lack in standardization and force the need of new classification criteria.
The criteria update should also improve the inclusion criteria for clinical trials. 


The proposed SICCA criteria have been developed and validated on 1618 participants in the SICCA registry showing signs or symptoms for SS.

For the classification of SS at least 2 of the 3 objective features should be met:
1. Serology: Positive anti-SSA/Ro and/or anti-SSB/La (or positive RF and ANA titer ≥1:320)
2. Labial salivary gland biopsy: focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm²
3. Ocular staining score ≥3 in keratoconjunctivitis sicca

Validation of the criteria and comparison to alternatives resulted in high sensitivity and specificity and showed high agreement with the American-European Consensus Group (AECG) criteria, used as gold standard.

Due to overlapping clinical features or interference with criteria tests individuals with following conditions should be excluded from Sjögren’s syndrome studies or therapeutic trials: History of head and neck radiation treatment, hepatitis C infection, acquired immunodeficiency syndrome, sarcoidosis, amyloidosis, graft versus host disease or IgG4-related disease.


These classification criteria were developed by the use of standardized measures and objective tests. They show improved classification performance compared to existing ones and thus reduce health risks through misclassification.


The newly developed classification criteria improve the diagnosis and management of Sjögren’s syndrome, as well as clinical trials on SS. The American College of Rheumatology (ACR) approved these Sjögren criteria as Provisional.

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As in all diagnostic testing, the diagnosis is made by the physican based on both test results and the patient history.