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Pathogenetic Aspects of Sjögren’s Syndrome: Relationships with Cryoglobulinemia and Lymphoproliferation of MALT

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Connective Tissue Disease

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Sjögren’s syndrome (SS) may be defined both as an autoimmune and a lymphoproliferative disease: in fact, B cells are overexpanded since the onset of the disease. SS is a disorder of mucosa-associated lymphoid tissue (MALT). The risk of B-cell lymphoma evolution is markedly increased in SS, this complication being observed in about 5 % of patients. A first pathogenetic trigger, such as an infection, may lead to local inflammation, which in turn stirs up an autoimmune process. The chronic stimulation of rheumatoid factor-positive B cells in the MALT microenvironment causes their preferential expansion in SS, leading to an increased risk for B-cell lymphoma. Cryoglobulinemia, with or without a concomitant cryoglobulinemic vasculitis, and the persistent swelling of the major salivary glands (usually the parotids) represent the two main risk factors for B-cell NHL.

Future studies focusing on SS-related B-cell lymphoproliferation may contribute to identify the key pathogenic events and to develop new therapeutic strategies in SS.

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Authors and Affiliations

  1. Department of Medical and Biological Science, Clinic of Rheumatology, University Hospital of “Santa Maria della Misericordia”, Piazza S. Maria della Misericordia 15, 33010, Udine, Italy

    Salvatore De Vita & Luca Quartuccio

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  1. Salvatore De Vita
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  2. Luca Quartuccio
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Correspondence to Salvatore De Vita .

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  1. Giovanni Bosco Hospital, Center of Immunopathology/Rare Dise, Torino, Torino, Italy

    Dario Roccatello

  2. University of Florence, Florence, Italy

    Lorenzo Emmi

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De Vita, S., Quartuccio, L. (2016). Pathogenetic Aspects of Sjögren’s Syndrome: Relationships with Cryoglobulinemia and Lymphoproliferation of MALT. In: Roccatello, D., Emmi, L. (eds) Connective Tissue Disease. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-24535-5_27

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  • DOI: https://doi.org/10.1007/978-3-319-24535-5_27

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