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Review
. 2013 Apr 4:11:93.
doi: 10.1186/1741-7015-11-93.

Novel aspects of Sjögren's syndrome in 2012

Angela Tincani  1 , Laura Andreoli, Ilaria Cavazzana, Andrea Doria, Marta Favero, Maria-Giulia Fenini, Franco Franceschini, Andrea Lojacono, Giuseppe Nascimbeni, Amerigo Santoro, Francesco Semeraro, Paola Toniati, Yehuda Shoenfeld
Affiliations
Review

Novel aspects of Sjögren's syndrome in 2012

Angela Tincani et al. BMC Med. .

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.

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Figures

Figure 1
Figure 1
Severe ocular surface damage in a dry eye patient.
Figure 2
Figure 2
Dry eye classification flow chart (Dry Eye Workshop Report, 2007) [19]. The causes of ‘dry eye syndrome’ are subdivided into aqueous-deficient and evaporative groupings. Sjögren’s syndrome (SS) belongs to the first group. Modified from [19].
Figure 3
Figure 3
Lissamine green staining of the conjunctiva in dry eye syndrome.
Figure 4
Figure 4
Microscopy of a minor salivary gland in Sjögren’s syndrome (SS): ‘periductal lymphoid focus’. The finding of at least 1 focus (periductal aggregate of at least 50 lymphocytes, mostly CD4+) in 4 mm2 of tissue is diagnostic for SS. The ‘score’ is the number of foci in 4 mm2 of tissue.
Figure 5
Figure 5
Microscopy of non-Hodgkin’s (NH) marginal B cell lymphoma. The most frequent lymphoma in Sjögren’s syndrome (SS) is the NH marginal B cell type, which comes from mucosa-associated lymphoid tissue (MALT).
Figure 6
Figure 6
Anti-CD20 staining of non-Hodgkin’s (NH) marginal B cell lymphoma. The lymphoid infiltrate is constituted by medium-sized cells, with a cleaved nucleus and a large cytoplasm, which are factors strongly positive for CD20.
See this image and copyright information in PMC

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