Sjögren Syndrome: An A-to-Z Update

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


December 03, 2013

In This Article

Clinical Manifestations

Although the genetics of different Sjögren syndrome groups show differences, the clinical manifestations in cohorts in the United States, Europe, China, and Japan are remarkably similar. The ESSDAI contains 12 domains that cover skin, lung, arthritis, neuropathy, and laboratory abnormalities; and Japanese patients exhibited similar clinical manifestations in each domain.[4]

However, it was surprising that the frequency of extraglandular manifestations, as measured by ESSDAI, was higher among Japanese patients in Japan than in American Japanese patients. Also, a lower ESSDAI score has been reported among patients of African descent with Sjögren syndrome. This seems contrary to our expectation of a lower ESSDAI score among a society that has a large vegetarian or fish intake as well as a national healthcare system for early diagnosis. Similarly, SLE among African-American patients has been reported as being significantly more severe.

A major difference in frequency of extraglandular symptoms may reflect ascertainment bias in the enrollment of patients. In Japan, China, and India, patients are first seen in a primary medical care clinic, and signs/symptoms such as rash, neuropathy, or major laboratory abnormalities are detected. These Japanese or Chinese patients are then referred to a rheumatology clinic where laboratory studies such as antibody to SS-A and lip biopsy are first ordered, the findings of which indicate Sjögren syndrome as the cause for the clinical manifestations.

This ascertainment bias of using clinical symptoms to document a clinical presentation is obvious in retrospect. In the United States, patients are more frequently screened for vague symptoms of fatigue with an ANA panel, and the finding of a positive serology leads to rheumatology evaluation.


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