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Eur J Oral Sci. 2018 Oct;126 Suppl 1:37-48. doi: 10.1111/eos.12536.

Current concepts on Sjögren's syndrome - classification criteria and biomarkers.

Author information

1
Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Bergen, Norway.
2
Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
3
Department of Clinical Dentistry - Section for Oral and Maxillofacial Radiology, University of Bergen, Bergen, Norway.
4
2C SysBioMed, Contra, Switzerland.
5
Gade Laboratory for Pathology, Department of Clinical Medicine, University of Bergen, Bergen, Norway.
6
Department of Pathology, Haukeland University Hospital, Bergen, Norway.

Abstract

Sjögren's syndrome is a lymphoproliferative disease with autoimmune features characterized by mononuclear cell infiltration of exocrine glands, notably the lacrimal and salivary glands. These lymphoid infiltrations lead to dryness of the eyes (keratoconjunctivitis sicca), dryness of the mouth (xerostomia), and, frequently, dryness of other surfaces connected to exocrine glands. Sjögren's syndrome is associated with the production of autoantibodies because B-cell activation is a consistent immunoregulatory abnormality. The spectrum of the disease extends from an organ-specific autoimmune disorder to a systemic process and is also associated with an increased risk of B-cell lymphoma. Current treatments are mainly symptomatic. As a result of the diverse presentation of the syndrome, a major challenge remains to improve diagnosis and therapy. For this purpose an international set of classification criteria for primary Sjögren's syndrome has recently been developed and validated and seems well suited for enrolment in clinical trials. Salivary gland biopsies have been examined and histopathology standards have been developed, to be used in clinical trials and patient stratification. Finally, ultrasonography and saliva meet the need of non-invasive imaging and sampling methods for discovery and validation of disease biomarkers in Sjögren's syndrome.

KEYWORDS:

Sjögren's syndrome; biomarkers; classification; histopathology; ultrasonography

PMID:
30178554
DOI:
10.1111/eos.12536

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