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Novel aspects of Sjögren’s syndrome in 2012

Angela Tincani12*, Laura Andreoli2, Ilaria Cavazzana1, Andrea Doria3, Marta Favero3, Maria-Giulia Fenini4, Franco Franceschini1, Andrea Lojacono5, Giuseppe Nascimbeni6, Amerigo Santoro7, Francesco Semeraro6, Paola Toniati1 and Yehuda Shoenfeld8

Author Affiliations

1 Rheumatology and Clinical Immunology Unit, Spedali Civili, Piazzale Spedali Civili 1, 25100 Brescia, Italy

2 Chair of Rheumatology, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Piazzale Spedali Civili 1, 25100 Brescia, Italy

3 Rheumatology Unit, Department of Medicine, University of Padua, Azienda Ospedaliera di Padova, Via Giustiniani 2, Padua, 35128, Italy

4 Rheumatology Unit, Vallecamonica Hospital, Via Manzoni 142, Esine (Brescia), 25040, Italy

5 Obstetrics and Gynecology Unit, Spedali Civili and University of Brescia, Piazzale Spedali Civili 1, Brescia, 25100, Italy

6 Ophthalmology Unit, Spedali Civili and University of Brescia, Piazzale Spedali Civili 1, Brescia, 25100, Italy

7 Pathology Unit, Spedali Civili, Piazzale Spedali Civili 1, Brescia, 25100, Italy

8 Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, Tel Hashomer, 52621, Israel

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BMC Medicine 2013, 11:93  doi:10.1186/1741-7015-11-93

Published: 4 April 2013

Abstract

Sjögren’s syndrome (SS) is a systemic progressive autoimmune disease characterized by a complex pathogenesis requiring a predisposing genetic background and involving immune cell activation and autoantibody production. The immune response is directed to the exocrine glands, causing the typical ‘sicca syndrome’, but major organ involvement is also often seen. The etiology of the disease is unknown. Infections could play a pivotal role: compared to normal subjects, patients with SS displayed higher titers of anti-Epstein-Barr virus (EBV) early antigens, but lower titers of other infectious agent antibodies such as rubella and cytomegalovirus (CMV) suggest that some infections may have a protective role against the development of autoimmune disease. Recent findings seem to show that low vitamin D levels in patients with SS could be associated with severe complications such as lymphoma and peripheral neuropathy. This could open new insights into the disease etiology. The current treatments for SS range from symptomatic therapies to systemic immunosuppressive drugs, especially B cell-targeted drugs in cases of organ involvement. Vitamin D supplementation may be an additional tool for optimization of SS treatment.

Keywords:
Anti-B cell therapies; Autoantibodies; Autoimmune diseases; Chronic sialoadenitis; Dry eye syndrome; Lymphoma; Peripheral neuropathy; Sicca syndrome; Sjögren’s syndrome; Vitamin D