The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives
There is a great deal of evidence pointing to interferons (IFNs) as being key cytokines in the pathogenesis of different systemic autoimmune diseases, including primary Sjögren’s syndrome (pSS). In this disease, a large number of studies have shown that an overexpression of type I IFN, the ‘so-calle...
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MDPI AG
2021-02-01
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| Series: | Biomolecules |
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| Online Access: | https://www.mdpi.com/2218-273X/11/2/251 |
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| author | Nicoletta Del Papa Antonina Minniti Maurizio Lorini Vincenzo Carbonelli Wanda Maglione Francesca Pignataro Nicola Montano Roberto Caporali Claudio Vitali |
| author_facet | Nicoletta Del Papa Antonina Minniti Maurizio Lorini Vincenzo Carbonelli Wanda Maglione Francesca Pignataro Nicola Montano Roberto Caporali Claudio Vitali |
| author_sort | Nicoletta Del Papa |
| collection | DOAJ |
| description | There is a great deal of evidence pointing to interferons (IFNs) as being key cytokines in the pathogenesis of different systemic autoimmune diseases, including primary Sjögren’s syndrome (pSS). In this disease, a large number of studies have shown that an overexpression of type I IFN, the ‘so-called’ type I IFN signature, is present in peripheral blood mononuclear cells, and that this finding is associated with the development of systemic extra-glandular manifestations, and a substantial production of autoantibodies and inflammatory cytokines. In contrast, the absence or a milder expression of type I IFN signature and low level of inflammatory cytokines characterizes patients with a different clinical phenotype, where the disease is limited to glandular involvement and often marked by the presence of widespread pain and depression. The role of type II (IFNγ) in this subset of pSS patients, together with the potentially related activation of completely different immunological and metabolic pathways, are emerging issues. Expression of both types of IFNs has also been shown in target tissues, namely in minor salivary glands where a predominance of type II IFN signature appeared to have a certain association with the development of lymphoma. In view of the role played by IFN overexpression in the development and progression of pSS, inhibition or modulation of IFN signaling has been regarded as a potential target for the therapeutic approach. A number of therapeutic compounds with variable mechanisms of action have been tested or are under consideration for the treatment of patients with pSS. |
| first_indexed | 2024-03-09T04:56:28Z |
| format | Article |
| id | doaj.art-b1b7a8152a274fbb899b710a4c15be02 |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-09T04:56:28Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-b1b7a8152a274fbb899b710a4c15be022023-12-03T13:05:34ZengMDPI AGBiomolecules2218-273X2021-02-0111225110.3390/biom11020251The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic PerspectivesNicoletta Del Papa0Antonina Minniti1Maurizio Lorini2Vincenzo Carbonelli3Wanda Maglione4Francesca Pignataro5Nicola Montano6Roberto Caporali7Claudio Vitali8Department of Rheumatology, ASST G. Pini-CTO, 20122 Milano, ItalyDepartment of Rheumatology, ASST G. Pini-CTO, 20122 Milano, ItalyDepartment of Clinical Sciences and Community Health, Ca’ Granda IRCCS Foundation, Ospedale Maggiore Policlinico, Università degli Studi di Milano, 20122 Milano, ItalyDepartment of Clinical Sciences and Community Health, Ca’ Granda IRCCS Foundation, Ospedale Maggiore Policlinico, Università degli Studi di Milano, 20122 Milano, ItalyDepartment of Rheumatology, ASST G. Pini-CTO, 20122 Milano, ItalyDepartment of Rheumatology, ASST G. Pini-CTO, 20122 Milano, ItalyDepartment of Clinical Sciences and Community Health, Ca’ Granda IRCCS Foundation, Ospedale Maggiore Policlinico, Università degli Studi di Milano, 20122 Milano, ItalyDepartment of Rheumatology, ASST G. Pini-CTO, 20122 Milano, ItalyMater Domini Humanitas Hospital, Rheumatology Outpatient Clinics, 21053 Castellanza, ItalyThere is a great deal of evidence pointing to interferons (IFNs) as being key cytokines in the pathogenesis of different systemic autoimmune diseases, including primary Sjögren’s syndrome (pSS). In this disease, a large number of studies have shown that an overexpression of type I IFN, the ‘so-called’ type I IFN signature, is present in peripheral blood mononuclear cells, and that this finding is associated with the development of systemic extra-glandular manifestations, and a substantial production of autoantibodies and inflammatory cytokines. In contrast, the absence or a milder expression of type I IFN signature and low level of inflammatory cytokines characterizes patients with a different clinical phenotype, where the disease is limited to glandular involvement and often marked by the presence of widespread pain and depression. The role of type II (IFNγ) in this subset of pSS patients, together with the potentially related activation of completely different immunological and metabolic pathways, are emerging issues. Expression of both types of IFNs has also been shown in target tissues, namely in minor salivary glands where a predominance of type II IFN signature appeared to have a certain association with the development of lymphoma. In view of the role played by IFN overexpression in the development and progression of pSS, inhibition or modulation of IFN signaling has been regarded as a potential target for the therapeutic approach. A number of therapeutic compounds with variable mechanisms of action have been tested or are under consideration for the treatment of patients with pSS.https://www.mdpi.com/2218-273X/11/2/251type I interferontype II interferonSjögren’s syndrometarget therapies |
| spellingShingle | Nicoletta Del Papa Antonina Minniti Maurizio Lorini Vincenzo Carbonelli Wanda Maglione Francesca Pignataro Nicola Montano Roberto Caporali Claudio Vitali The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives Biomolecules type I interferon type II interferon Sjögren’s syndrome target therapies |
| title | The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives |
| title_full | The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives |
| title_fullStr | The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives |
| title_full_unstemmed | The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives |
| title_short | The Role of Interferons in the Pathogenesis of Sjögren’s Syndrome and Future Therapeutic Perspectives |
| title_sort | role of interferons in the pathogenesis of sjogren s syndrome and future therapeutic perspectives |
| topic | type I interferon type II interferon Sjögren’s syndrome target therapies |
| url | https://www.mdpi.com/2218-273X/11/2/251 |
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