Evolving concepts of diagnosis, pathogenesis, & therapy of Sjogren’s syndrome

Differences in diagnostic criteria for Sjogren's Syndrome (SS) have
led to confusion in the research literature and in clinical
practice. A particular challenge is the clinical diagnosis of
the patients with sicca symptoms, fibromyalgia, chronic
fatigue, vague cognitive defects, and a low titer antinuclear
antibody. Until recently, many of these patients would have
been classified as primary SS using the European criteria. A
suggested revision of the European criteria will require
inclusion of anti SS-A antibody or characteristic minor
salivary gland biopsy, leading to greater agreement between
European and San Diego criteria. Recent studies have
emphasized that lacrimal and salivary gland flow involves an
entire "functional" unit that includes the mucosal surface
(the site of inflammation), efferent [?afferent] nerve signals
sent to the midbrain (lacrimatory and salvatory nucleus),
efferent neural signals from the brain, and acinal/ductal
structures in the gland. Thus, symptoms of dryness or pain can
result from interferences with any part of this functional
unit. The initiating antigens in SS remain unknown, but immune
reactivity against SS-A, SS-B, fodrin, alpha amylase, and
carbonic anhydrase have been demonstrated in patients with
established disease. The inflammatory process in the gland
releases metalloproteinases that alter the relationship of
epithelial cells to their matrix, an interaction that is
necessary for glandular function and survival. Therapies for
SS remain inadequate. In SS patients with immune-mediated
extraglandular manifestation (ie, lung, kidney, skin, nerve),
the therapeutic approach his similar to systemic lupus
erythematosus, although these therapies have relatively little
effect on tear or saliva flow.

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