Progress in Classifying and Managing Sjögren’s Syndrome

VBCR - February 2013, Volume 2, No 1 - Autoimmune Diseases

Washington, DC—Sjögren’s syndrome has been understudied and is not well understood, but an international collaboration has produced new classification criteria for this autoimmune disease, and experts are working on guidelines for the management of this condition. These efforts were discussed at the 2012 meeting of the American College of Rheumatology (ACR).

The New Criteria
New criteria for the classification of Sjögren’s syndrome proposed by the ACR were developed by an international group called the Sjögren’s Syndrome International Collaborative Clinical Alliance (SICCA) and were based on 1362 participants who were enrolled in the SICCA registry. The proposed classification considers that Sjögren’s syndrome is a multisystem autoimmune disease that should involve multidisciplinary treatment in rheumatology, ophthalmology, and oral medicine.

Rheumatologists are supposed to be experts in the diagnosis and management of Sjögren’s syndrome, explained Lindsey A. Criswell, MD, MPH, Chief of the Division of Rheumatology at the University of California, San Francisco, and coauthor of the new ACR criteria. “Yet the under-­attention it has received in terms of research has been a major problem for patients with Sjögren’s syndrome.”

Dr Criswell commented that the new criteria, which are based on data from the SICCA registry, will address this gap by enabling improved research into the etiology, genetics, and therapy of this autoimmune disease. These criteria are stringent enough to be used as entry criteria for clinical trials of new biologic immunomodulating agents.

Over the past 4 decades, a variety of approaches to diagnosing or classifying patients with Sjögren’s syndrome have been proposed. However, none has been endorsed by the ACR or the European League Against Rheumatism, and there has been no consensus on the approach to classification, Dr Criswell stated.

The new criteria encompass the 3 main components of the syndrome:

  • Dryness and other effects in the eyes
  • Dryness and other effects in the mouth
  • ystemic manifestations.

The proposed classification is based on objective test results, and patients must meet 2 of the 3 following objective features to be classified with Sjögren’s syndrome:

  1. A positive serum anti-SSA and/or anti-SSB result, or a positive rheumatoid factor and antinuclear antibody titer >1:320
  2. An ocular staining score >3
  3. The presence of focal lymphocytic sialadenitis with a focus score >1 focus/4 mm2 in labial salivary gland biopsy samples.

The authors of the proposed classification criteria said that previous diagnoses of certain conditions would be considered exclusions for participation in trials because of overlapping clinical criteria, including history of head-and-neck radiation treatment, hepatitis C infection, AIDS, sarcoidosis, amyloidosis, graft-versus-host disease, and immunoglobulin G4–related disease.

The proposed Sjögren’s criteria do away with the distinction between primary and secondary forms of the disease, considering this distinction to be most likely obsolete. “It seems of little use and risks potential confusion to distinguish in a given patient one autoimmune disease and secondary to another. Accordingly, the diagnosis should be given to all who fulfill these criteria while diagnosing any concurrent organ-specific or multiorgan automimmune diseases, without distinguishing as primary and secondary,” the authors of the criteria wrote.

Guideline Development
Guidelines for the management of Sjögren’s syndrome are currently being developed under the umbrella of the Sjögren’s Syndrome Foundation, said Steven E. Carsons, MD, Chief of the Division of Rheumatology, Clinical Immunology, and Allergy at Winthrop-University Hospital, Mineola, New York.
The committee developing the guidelines is addressing 3 main issues:

  • Which treatments are beneficial for the treatment of inflammatory joint disease associated with Sjögren’s syndrome?
  • What is the appropriate management of fatigue?
  • What is the role of oral and biologic disease-modifying antirheumatic drugs for the SICCA syndrome components of Sjögren’s syndrome?

“These areas represent a significant unmet need,” Dr Carsons said.

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