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Primary Sjögren's syndrome and the type I interferon system
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Rheumatology. (Systemic Autoimmunity)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Rheumatology. (Systemic Autoimmunity)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Rheumatology. (Systemic Autoimmunity)
2012 (English)In: Current Pharmaceutical Biotechnology, ISSN 1389-2010, E-ISSN 1873-4316, Vol. 13, no 10, 2054-2062 p.Article, review/survey (Refereed) Published
Abstract [en]

Patients with primary Sjögren's syndrome (pSS) have an activated type I interferon (IFN) system that contribute to the etiopathogenesis and clinical manifestations of the disease. The type I IFN system consists of the stimuli for type I IFN production, the receptors, cells and transcription factors involved in the synthesis of type I IFNs, the type I IFN-receptor and the effects on target cells. Increased type I IFN activity has been demonstrated in sera from patients with pSS and IFN-α, the main type I IFN, has been detected in the minor salivary glands. Gene expression profiling of peripheral blood mononuclear cells (PBMCs) and minor salivary glands from pSS patients display an up-regulation of type I IFN-induced genes, an "IFN signature". The professional IFN-α producing plasmacytoid dendritic cell (pDC) shows a reduced frequency in the peripheral blood, but has been detected in the salivary glands, possibly due to tissue recruitment. Polymorphisms in the interferon regulatory factor 5 (IRF5) and signal transducer and activator of transcription 4 (STAT4) genes in the type I IFN system, are associated with increased risk for pSS. A postulated disease model is that an initial viral infection induces type I IFN production in the salivary glands with subsequent activation of the adaptive immune system resulting in the production of autoantibodies against the RNA-binding proteins SSA/SSB/RNP. Interferogenic immune complexes are formed, which trigger the pDCs to an ongoing type I IFN production, which sustain the disease process. Potential therapeutic targets can be identified within the type I IFN system.

Place, publisher, year, edition, pages
2012. Vol. 13, no 10, 2054-2062 p.
Keyword [en]
Immune complexes, interferon-alpha, IRF5, minor salivary glands, plasmacytoid dendritic cells, primary Sjogren's syndrome, SSA/SSB, STAT4, type I interferon
National Category
Medical and Health Sciences
Research subject
Medicine; Medicine
Identifiers
URN: urn:nbn:se:uu:diva-165331ISI: 000306835200024PubMedID: 22208657OAI: oai:DiVA.org:uu-165331DiVA: diva2:473152
Available from: 2012-01-05 Created: 2012-01-05 Last updated: 2017-12-08Bibliographically approved

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Nordmark, GunnelEloranta, Maija-LeenaRönnblom, Lars

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