Are the New Sjögren Syndrome Criteria Necessary?

Robert I. Fox, MD, PhD


September 18, 2012

The New Sjögren Syndrome Criteria: A Summary

A Data-Driven, Expert Consensus Approach to a Classification Criteria for Sjögren Syndrome: International Collaborative Clinical Alliance Cohort

Shiboski and colleagues[1] propose new classification criteria for Sjögren syndrome (SS) because of the emergence of biologic agents as potential treatments and their associated comorbidity. The new criteria are based on expert opinion elicited from the nominal group technique and analyses of data from the Sjögren's International Collaborative Clinical Alliance (SICCA). The new criteria were accepted by the American College of Rheumatology (ACR), according to the National Institutes of Health Newsletter[2]on April 11, 2012, but they have not been accepted in Europe (ie, EULAR, European League Against Rheumatism).

SICCA believes that new classification criteria for SS are needed to better support etiologic and genetic research and therapeutic trials for this prevalent autoimmune disease. The group also emphasizes that criteria used for enrollment into clinical trials need to be clear, easy to apply, and have high specificity, considering the potentially serious adverse effects and comorbidities of biologic agents.

This relatively sudden acceptance of new ACR criteria for SS puts the practicing rheumatologist in a quandary. The issue was discussed at the last EULAR annual meeting and will be further discussed at the next ACR meeting among participants from major US medical institutions with SS treatment centers (collectively referred to as the SS Task Workgroup).

I believe that ACR's recognition of the SICCA criteria as the sole criteria for SS may be premature for reasons that are discussed below. I propose that additional statistical review is necessary before the SICCA criteria are considered valid to replace the American-European Consensus Group (AECG) criteria. It is my understanding that serious concerns about statistical validation of the SICCA protocol were discussed at the EULAR meeting, although the specifics have not yet been published. A committee has been appointed to resolve these issues. Hopefully, the upcoming ACR meeting will provide more insight on the status of this discussion.

SICCA criteria specify that a diagnosis of SS requires 2 of the following:

  • Positive serum anti-SSA and/or anti-SSB (or positive rheumatoid factor and antinuclear antibody titer > 1:320);

  • Ocular staining score > 3;

  • Presence of focal lymphocytic sialadenitis with a focus score > 1 focus/4 mm3 in labial salivary gland biopsy samples.

The proposed criteria by Shiboski and colleagues differ from the currently used AECG ones by not requiring symptoms that have been used previously for both glandular manifestations (ocular and oral dryness).