According to Alan N. Baer, M.D., of Johns Hopkins University, Baltimore, a team effort is required to arrive at a diagnosis of Sjögren's syndrome, a chronic inflammatory autoimmune disorder involving dryness of the eyes, mouth, and other mucous membrane tissue.  The syndrome often occurs with other autoimmune diseases, e.g., lupus, polymyositis, and rheumatoid arthritis.  Dr. Baer suggests that a rheumatologist or primary care physician working alone cannot provide the sort of diagnostic accuracy that he has in mind.

     Dr. Baer says that there is clearly the need for an ophthalmologist, a good oral pathologist, and ideally an oral surgeon to do the biopsy.  He suggests that a Sjögren's-savvy neurologist can be a big help, too, since peripheral neuropathies occur in 20 percent of Sjögren's patients.  According to Dr. Baer, neuropathic symptoms often precede diagnosis of the rheumatic disorder.  Many pathologists are not skilled in interpreting labial gland biopsies.  Dr. Baer points out that this is a big problem in the diagnosis of Sjögren's.

     Although the Schirmer's test is still widely used for objective documentation of the ocular component of Sjögren's, corneal and conjunctival staining and/or tear breakup time are better measures of the severity of dry eyes, according to Dr. Baer.

     The lifetime lymphoma risk in patients with Sjögren's syndrome is roughly 16-fold greater than that of the general population; and in a study of nearly 30,000 patients with various autoimmune disorders, Sjögren's syndrome stood out as having far and away the highest risk of non-Hodgkin's lymphoma.

     In the meantime, a National Institutes of Health (NIH)-funded international project is underway with the goal of developing simplified, standardized, reliable, and more clinically relevant classification criteria for Sjögren's syndrome.  Utilizing the Sjögren's International Collaborative Clinical Alliance (SICCA) registry headquartered at the University of California, San Francisco, researchers have amassed detailed clinical data and biospecimens from more than 1,600 patients who range across the full spectrum from possible early Sjögren's syndrome to well-established advanced disease.

     Dr. Baer observes that, to date, there is a dearth of convincing clinical evidence supporting the efficacy of immunomodulatory therapies, including hydroxychloroquine, widely considered the first-line drug.  A number of experts feel that some of the biologic therapies which have had a major impact in other rheumatic diseases have promise in Sjögren's syndrome.  However, their successful application will require early identification of affected patients and reliable markers of disease activity, which the SICCA registry aims to provide.

     NIH officials have committed to at least a decade of SICCA funding.  Dr. Baer notes that the SICCA registry already has borne fruit in the form of a recently published simplified quantitative means for diagnosing the ocular component of Sjögren's syndrome.

--Sources: "Diagnosing Sjögren's Syndrome Requires a Multispecialty Effort" and "Major NIH Project Aims to Improve Sjögren's Diagnosis, Bruce Jancin, Rheumatology News, April 2010