{"title":"Autoantibodies and autoantigens in Sjögren’s syndrome","authors":"A. Ambrosi, A. Björk, M. Wahren-Herlenius","doi":"10.1093/MED/9780198806684.003.0005","DOIUrl":"https://doi.org/10.1093/MED/9780198806684.003.0005","url":null,"abstract":"Autoantibodies are a key serological feature of Sjögren’s syndrome (SS). The presence of Ro/Sjögren’s syndrome-related antigen A (SSA) autoantibodies is one of the items with the highest weight in the 2016 joint ACR/EULAR SS classification criteria. Autoantibodies appear before overt clinical disease manifestations, and patient autoantibody profiles seem stable over time, even after B-cell depleting therapy. Expression of Ro/SSA and La/Sjögren’s syndrome-related antigen B (SSB), the major autoantigens in SS, in the target organs (exocrine glands), local autoantibody production, and the capacity of autoantigen-containing immune complexes to induce interferon production all point to a central involvement of autoantibodies in disease pathogenesis. Here, we review the main autoantibody specificities reported in SS, their clinical associations, the current understanding of how autoantibody production is initiated and maintained, and how autoantibodies may exert pathogenic effects. We provide a comprehensive overview of the nature and biological function of the three main autoantigens, Ro52, Ro60, and La (Ro/SSA and La/SSB) found in SS.","PeriodicalId":260788,"journal":{"name":"Oxford Textbook of Sjögren's Syndrome","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130833838","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Sjögren’s syndrome–associated lymphoma","authors":"S. Rassam","doi":"10.1093/MED/9780198806684.003.0025","DOIUrl":"https://doi.org/10.1093/MED/9780198806684.003.0025","url":null,"abstract":"Non-Hodgkin’s lymphoma (NHL), predominantly of the B-cell type, affects a significant proportion of patients with Sjögren’s syndrome (SS). SS carries the highest NHL risk among all systemic autoimmune diseases. Although marginal zone lymphomas (MZL) of the mucosa-associated lymphoid tissue (MALT) type of the salivary glands is the commonest histological type, other indolent and more aggressive subtypes can occur. NHL and its treatment adds to the morbidity and mortality in SS. Early detection and management can improve survival and quality of life in SS patients. This chapter will review some basics of the immune system; expand on NHL incidence and predictive risk factors, pathogenesis, and subtypes of NHL seen in SS; clinical presentation; diagnosis; management; treatment modalities; and complications of therapy. Recent advances in therapy using our expanding knowledge of the molecular pathways seen in NHL will be discussed, as well as novel molecules and innovative immunotherapy.","PeriodicalId":260788,"journal":{"name":"Oxford Textbook of Sjögren's Syndrome","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125143074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Nonpharmacological interventions for the management of fatigue and constitutional symptoms in Sjögren’s syndrome","authors":"K. Hackett, H. Cartner","doi":"10.1093/MED/9780198806684.003.0023","DOIUrl":"https://doi.org/10.1093/MED/9780198806684.003.0023","url":null,"abstract":"Current treatments for Sjögren’s syndrome (SS) are limited. Pharmacological treatments tend to focus on topical treatments for dryness or oral muscarinic agonists which increase tear production and salivary flow. National treatment guidelines for primary SS incorporating available published evidence and expert opinion include recommendations for specific pharmacological topical and systemic treatments. However, despite the availability of such treatments, SS patients still experience symptoms, difficulties performing their daily activities, and reduced quality of life. This chapter explores specific ways nonpharmacological support for patients with primary SS might be delivered. These include the provision of information and patient education, as well as specific symptom management support for anxiety, sleep disturbances, pain, discomfort, low mood, and anxiety. Possible modes of delivering interventions and consideration of the differing levels of support which are required for different patients are also discussed.","PeriodicalId":260788,"journal":{"name":"Oxford Textbook of Sjögren's Syndrome","volume":"24 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132709514","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Renal involvement in primary Sjögren’s syndrome","authors":"R. Evans, S. Walsh","doi":"10.1093/med/9780198806684.003.0017","DOIUrl":"https://doi.org/10.1093/med/9780198806684.003.0017","url":null,"abstract":"Renal involvement in primary Sjögren’s syndrome (pSS) was first described in the 1960s, with reports of acquired defects in tubular function, in the setting of lymphocytic tubulointerstitial inflammation. Renal involvement in pSS is common, but is frequently undiagnosed due to its covert nature. It may manifest either as epithelial disease causing tubulointerstitial nephritis, or as an immune-complex glomerulopathy. Tubulointerstitial inflammation leads to acquired defects in tubular function, which may occur at multiple nephron segments. Treatment protocols lack evidence from randomized trials, and involve immunosuppression targeting B- and T-cells, which mediate both interstitial and glomerular disease to varying extents. In this chapter, we review the prevalence of renal involvement in pSS and its clinical manifestations, likely pathogenesis, and treatment.","PeriodicalId":260788,"journal":{"name":"Oxford Textbook of Sjögren's Syndrome","volume":"71 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117303607","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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