Sjögren Syndrome: An A-to-Z Update

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


December 03, 2013

In This Article

Summary and Conclusions

There was great interest in the application of ultrasound for the diagnosis of Sjögren syndrome and as a noninvasive method to follow therapy. However, interobserver variations and technical standardization will be required before this can be used in the hands of practicing rheumatologists.

Systemic biologic therapy with abatacept and belimumab showed benefit in small uncontrolled studies of patients with early disease. Rituximab continues to be the most widely used biologic in patients with systemic manifestations and is used in response to acute flares such as vasculitis or parotid swelling. However, "benign symptoms" of oral and ocular discomfort showed little consistent improvement even in patients with systemic (ESSDAI) improvement.

The most exciting highlights of this year's ACR were in the field of genetics of Sjögren syndrome. We obtained genetic confirmation of previous cytokine profiles from glandular biopsies that both innate and acquired immune systems were involved. A difference between Sjögren syndrome and SLE was found in the GWAS (CXCR5), an observation that makes clinical sense to explain the "homing" and thus lymphoproliferative properties in Sjögren syndrome that are not present in SLE. Also suggested by genomic studies, NK cells (NKp3/B7H6) may provide a link between acquired and innate immune systems. Therefore, a new set of therapeutic targets may emerge.

Pharmaceutical companies expressed an interest in Sjögren syndrome and are exploring a series of biologics and small-molecule inhibitors. In a decade that has seen multiple drugs approved for treatment of multiple sclerosis, we have yet to have a good therapy for Sjögren syndrome.

Differences in classification criteria (AECG and SICCA) must be resolved quickly because they are leading to confusion among clinicians, researchers, and the pharmaceutical industry. Even though the 2 sets of criteria are 90% concordant, the 10% of discordant patients could influence the outcome of ESSDAI scores and clinical trials. Neither set of criteria is currently satisfactory, and a consensus should incorporate the best of each proposal before official approval is granted by the ACR.


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