Title:Current and Future Challenges in Primary Sjogren’s Syndrome
Volume: 13
Issue: 10
Author(s): Arjan Vissink, Hendrika Bootsma, Fred KL Spijkervet, Shen Hu, David T Wong and Cees GM Kallenberg
Affiliation:
Keywords:
B cell depletion therapy, biologicals, disease activity, disease progression, intervention therapy, pathophysiology, saliva, Sjogren’s syndrome, autoimmune inflammatory, exocrine glands, malaise and fatigue, symptoms, marginal zone B-cell lymhomas, salivary diagnostics.
Abstract: Sjogren’s syndrome (SS) is an autoimmune inflammatory disorder of exocrine glands. SS particularly affects
the lacrimal and salivary glands. Dry mouth and dry eyes are frequently proffered as presenting symptoms, but nonspecific
symptoms such as malaise and fatigue, and extraglandular manifestations like purpura, polyneuropathy and arthritis
are also often present. Moreover, lymphomas develop in about 7.5% of SS patients, mostly marginal zone B-cell lymhomas.
Futhermore, SS has a very substantial impact on the patients’ quality of life and their daily activities. Recently,
many breakthroughs have been seen in salivary diagnostics, which not only can be used for diagnosis but also for monitoring
of disease activity and disease progression as well as for objectively scoring the effect of intervention treatment with
biologicals. In addition, salivary proteomics, genomics and system biology have been shown to be very promising tools in
unravelling the pathophysiology of SS, thus providing in depth insight in its underlying mechanisms which could give
clues for intervention therapies with biologicals. The latter is of particular interest as B cell depletion therapy has been
shown a very promising therapy for a subgroup of SS patients. When applying salivary diagnostics in combination with
instruments to rate disease activity and progression in SS, one might be able to select those SS patients who respond to a
particular type of biological. These topics are addressed in this review and promises for the near future are described.
