Reconciling a Sjögren's Diagnosis: Worth the Effort?

Robert I. Fox, MD, PhD


February 08, 2013

In This Article


The American College of Rheumatology (ACR)/Associate Rheumatology Health Professional (AHRP) 2012 Annual Meeting was held in Washington, DC, in November. In addition to the usual presentations on therapy and pathogenesis, this year's meeting included discussions[1,2] on how to reconcile the American-European Consensus Group (AECG) criteria[3] for Sjögren syndrome (SS) -- the most commonly used clinical criteria for SS -- with the ACR-Sjögren's International Collaborative Clinical Alliance (SICCA) criteria,[4] both of which have been provisionally accepted by the ACR as official SS criteria.

The provisional acceptance by the ACR was conditional on validation in an independent cohort of patients,[5] and several symposia were devoted to the validation process. In addition to debates on the SS criteria, presentations regarding early diagnosis, pathogenesis, and therapy were also given (abstracts are available online).

Controversies in Diagnosis

Primary SS (pSS) is generally defined as an autoimmune connective tissue disease that typically affects the salivary and lacrimal glands, with consequently reduced saliva and tear production, as well as extraglandular manifestations. Patients with pSS have increased risk for lymphocytic infiltrative involvement of other organs, including B-cell lymphoma, neuropathies, interstitial pneumonitis, and nephritis. SS overlaps with other autoimmune disorders, in particular systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), but it has proven important to recognize pSS as a distinct entity to draw attention to the particular therapeutic needs, extraglandular complications, and prognostic features of patients with SS.

In a special session of the ACR concurrent meeting, Steven Shiboski (University of California, San Francisco), who is the lead author of the ACR-SICCA article,[5] reviewed the criticisms and unsolved problems of the previously used AECG classification criteria:

• Nonequivalent tests are considered to be equivalent in some domains (for instance, the Schirmer test and dye score in the ocular domain);

• Some invasive and expensive tests are included in the AECG criteria set (eg, sialography and salivary scintigraphy);

• Some tests, such as sialography, are now considered obsolete;

• The ACR-SICCA criteria methodology was similar to that used by the ACR for the 1987 RA criteria, whereas the AECG criteria have not been accepted by the ACR owing to lack of validation in the United States (although they were previously validated in Europe);

• Statistical comparison between the AECG and SICCA criteria showed strong agreement (kappa value, 0.88); and

• The ACR-SICCA criteria require 2 out of 3 positive criteria: anti-SS-A or SS-B antibody or positive antinuclear antibody (ANA) titer > 1:320, plus a positive rheumatoid factor (RF) titer; ocular staining score ≥ 3; or a positive labial gland biopsy with focus score ≥ 1.