Sjögren Syndrome: An A-to-Z Update

Robert I. Fox, MD, PhD; Carla M. Fox, RN


December 03, 2013

In This Article

Differences in the Criteria

The SICCA criteria differ from the 2002 AECG criteria in 3 ways:

They include no subjective ocular and oral symptoms and no functional or morphologic tests for the salivary glands;

They use a new ocular staining score as the only criterion for ocular involvement; and

They allow the use of an antinuclear antibody (ANA) titer ≥ 1:320 plus rheumatoid factor positivity as an alternative to anti-SSA/SSB antibody positivity for the assessment of systemic autoimmunity.

Reports from several cohorts that compared SICCA and AECG classification have recently been published, and their results were further discussed at the meeting. These included the Oklahoma cohort (Scoffield-United States), the Scandinavian cohort (Jonsson-Norway), the French Brest Cohort (Devic-France) and the Japanese cohort (Sumida-Japan).

In particular, the use of unstimulated saliva flow in AECG (not required in SICCA) helped with specificity of diagnosis of Sjögren syndrome. Furthermore, the finding of low Schirmer test (AECG) did not closely correlate with ocular staining scores >3 (SICCA) and contributed to differences between classification criteria. Although small in number, the patients who are discordant in the 2 criteria may lead to confusion in classification because currently used endpoints (such as the ESSDAI) for clinical studies have been based on the AECG criteria.[7]

Several conclusions can be derived from these discussions:

The SICCA criteria have been called the ACR criteria because they were granted ACR recognition contingent on validation by an independent cohort.

   −The site chosen for the external validation (Xavier-Paris) announced that the grant proposal to support this validation was not approved; therefore, no validation is on the immediate horizon.

   −Because this validation has not been performed, European participants (Jonsson-Norway; Cornec-France; Vitali-Italy) indicated that the criteria should still be referred to as the SICCA criteria (not the ACR criteria).

According to all participants, significant changes in the SICCA criteria will be required before they are ready for the required validation.

   −The SICCA criteria are now listed on the ACR Website with the implication that they are the only acceptable criteria, even though they have not been validated and will require modification.

   −Although it sounds like a trivial semantic issue, there are already reports of research grants and treatment protocols being rejected as a result of the AECG criteria being used rather than the SICCA criteria.

For the present time, we should enroll patients in clinical studies that fulfill both AECG and SICCA criteria.

   −This is important because both clinicians and regulatory agencies have familiarity with AECG criteria and the ESSDAI method of assessing extraglandular activity.

All participants agreed that there is a pressing need to overcome the current differences in diagnostic criteria that are generating unnecessary diversion from our real task of developing effective therapy.


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