COMMENTARY

Advances in Understanding, Diagnosing, and Treating Sjogren Syndrome

Robert I. Fox, MD, PhD

Disclosures

July 31, 2015

In This Article

Introduction

Sjogren syndrome (SS) was named for Henrik Sjögren, a Swedish ophthalmologist who, in 1933, reported the clinical and histologic details of 19 women with xerostomia and keratoconjunctivitis in his doctoral dissertation. Of these patients, 12 had associated arthritis. His findings remained largely ignored until Bloch and colleagues "rediscovered" SS in 1956.[1] Since this early research, there has been debate over the clinical criteria for diagnosis, the optimal methods of diagnosis, and modalities of therapy.

The first International Symposium on SS was held in 1986, organized by Drs Rolf Manthorpe and Peter Oxholm. Henrik Sjögren was supposed to attend, but unfortunately, last minute illness precluded us from meeting him. The symposium was attended by a few dozen faithful clinicians and investigators (myself included) from multiple disciplines, including oral medicine, ophthalmology, and hematology.

The 13th International Symposium on SS was held in Bergen, Norway, on May 19-22, 2015, and was organized by Dr Roland Jonsson, who also participated in the first symposium. This year's meeting had several hundred delegates from four continents and presented more than 200 abstracts, with more details on the meeting's website.[2] One of the exciting features of this year's meeting was the "passing of the torch" to a new generation of clinicians and researchers by the original group of "old timers" (myself included).

Some highlights from the original research presented include:

  • Agreement on consensus diagnostic criteria that will allow uniform diagnosis of patients;

  • Guidelines for evaluating efficacy of therapy that will be the basis of clinical trials, in particular a weighted scale that will be analogous to the American College of Rheumatology (ACR) ACR50 score for rheumatoid arthritis (RA);

  • Advances in methodology including parotid ultrasound and biomarkers that will provide noninvasive methods for diagnosis and determining prognosis and response to treatment;

  • Application of new pathogenetic tools to cohorts of SS patients including genome-wide association (GWA) studies that suggest important therapeutic targets;

  • Recognition that dynamic alterations beyond the genome play a role including alterations in DNA methylation profiles and microRNA transcription;

  • Translational studies to understand the high incidence of lymphoma and pathogenesis of extraglandular markers at the molecular level; and

  • Further advances in the complex pathways of cytokines and chemokines not only in blood samples but also in lacrimal and salivary secretions.

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