Advances in Understanding, Diagnosing, and Treating Sjogren Syndrome

Robert I. Fox, MD, PhD


July 31, 2015

In This Article

New Diagnostic Criteria

Caroline and Stephen Shiboski, on behalf of the ACR-European Union League Against Rheumatism (EULAR) Working Group, presented consensus diagnostic criteria for SS based on expert opinion and rigorous validation ( Table ).[3] The development of consensus criteria had been diligently pursued by Dr Troy Daniels and Dr John Greenspan, who were key participants in the original SS symposium.

The new consensus criteria should be published in the near future, expanding on prior publications.[4,5,6,7] Oral and ocular symptoms were defined and should be present as objective criteria for SS to be considered. However, extraglandular symptoms suggestive of SS, such as mixed cryoglobulinemia, renal distal tubular acidosis, pulmonary forms of lymphocytic interstitial pneumonitis, neurologic manifestations including neurologic myelitis ophthalmica, and/or lymphadenopathy (involving parotid glands) should also be considered, as these may be dominant over the patient's sicca complaints.

Indeed, it became clear in several presentations that many of the sickest SS patients are misdiagnosed with systemic lupus erythematosus (SLE) or RA, particularly in nonrheumatology settings. To confirm the diagnosis of SS, objective criteria including tear and saliva flow, autoantibody status including SS-A, and minor salivary gland histology are needed. We have all seen patients with multiple signs and symptoms of SS but who have a negative or nondiagnostic minor salivary gland biopsy. These patients can also be diagnosed using the weighted criteria (Table). Patients must have eye and oral symptoms and a score of 4 to be diagnosed with SS.

Issues with misdiagnoses in SS were illustrated in a study by Rasmussen and colleagues,[8] which examined a large cohort of patients that had been previously diagnosed with RA or SLE in their internal medicine clinic (n=531). They found that 75.5% of these patients did not meet criteria for either RA or SLE but that 45.6% fulfilled criteria for primary SS. Thus, we might suppose that many of the sicker patients with extraglandular manifestations (including those outside of rheumatology settings) actually have primary SS.

The search for patients with SS and their inclusion in future trials of SS treatment will have a great impact on outcomes. Currently, we are likely not including our sickest SS patients due to diagnostic misclassification. The inclusion of a substantially larger proportion of patients with extraglandular manifestations such as hemolytic anemia, thrombocytopenia, mixed cryoglobulinemia, objective arthritis, and vasculitis might dramatically change the outcomes in previously published trials on several biologics, such as rituximab.[9,10,11,12] Thus, we may need to re-examine several prior drug trials that concluded that the improvement in SS patients was not statistically significant because these trials did not include sufficient numbers of patients with extraglandular manifestations.


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