The epidemiology of Sjögren’s syndrome
Ruchika Patel, Anupama Shahane Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA Abstract: Sjögren’s syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It can present as an entity by itself, pr...
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| Format: | Article |
| Language: | English |
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Dove Medical Press
2014-07-01
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| Series: | Clinical Epidemiology |
| Online Access: | http://www.dovepress.com/the-epidemiology-of-sjoumlgrenrsquos-syndrome-peer-reviewed-article-CLEP |
| _version_ | 1818528996982259712 |
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| author | Patel R Shahane A |
| author_facet | Patel R Shahane A |
| author_sort | Patel R |
| collection | DOAJ |
| description | Ruchika Patel, Anupama Shahane Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA Abstract: Sjögren’s syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It can present as an entity by itself, primary Sjögren's syndrome (pSS), or in addition to another autoimmune disease, secondary Sjögren's syndrome (sSS). pSS has a strong female propensity and is more prevalent in Caucasian women, with the mean age of onset usually in the 4th to 5th decade. Clinical presentation varies from mild symptoms, such as classic sicca symptoms of dry eyes and dry mouth, keratoconjunctivitis sicca, and xerostomia, to severe systemic symptoms, involving multiple organ systems. Furthermore, a range of autoantibodies can be present in Sjögren's syndrome (anti-SSA/Ro and anti-SSB/La antibodies, rheumatoid factor, cryoglobulins, antinuclear antibodies), complicating the presentation. The heterogeneity of signs and symptoms has led to the development of multiple classification criteria. However, there is no accepted universal classification criterion for the diagnosis of Sjögren's syndrome. There are a limited number of studies that have been published on the epidemiology of Sjögren's syndrome, and the incidence and prevalence of the disease varies according to the classification criteria used. The data is further confounded by selection bias and misclassification bias, making it difficult for interpretation. The aim of this review is to understand the reported incidence and prevalence on pSS and sSS, the frequency of autoantibodies, and the risk of malignancy, which has been associated with pSS, taking into account the different classification criteria used. Keywords: Sjögren's syndrome, incidence, prevalence, classification criteria, autoantibodies, lymphoma |
| first_indexed | 2024-12-11T06:57:21Z |
| format | Article |
| id | doaj.art-8829e1e955c749b2a4d279ff34d809e0 |
| institution | Directory Open Access Journal |
| issn | 1179-1349 |
| language | English |
| last_indexed | 2024-12-11T06:57:21Z |
| publishDate | 2014-07-01 |
| publisher | Dove Medical Press |
| record_format | Article |
| series | Clinical Epidemiology |
| spelling | doaj.art-8829e1e955c749b2a4d279ff34d809e02022-12-22T01:16:44ZengDove Medical PressClinical Epidemiology1179-13492014-07-012014default24725517774The epidemiology of Sjögren’s syndromePatel RShahane ARuchika Patel, Anupama Shahane Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA Abstract: Sjögren’s syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It can present as an entity by itself, primary Sjögren's syndrome (pSS), or in addition to another autoimmune disease, secondary Sjögren's syndrome (sSS). pSS has a strong female propensity and is more prevalent in Caucasian women, with the mean age of onset usually in the 4th to 5th decade. Clinical presentation varies from mild symptoms, such as classic sicca symptoms of dry eyes and dry mouth, keratoconjunctivitis sicca, and xerostomia, to severe systemic symptoms, involving multiple organ systems. Furthermore, a range of autoantibodies can be present in Sjögren's syndrome (anti-SSA/Ro and anti-SSB/La antibodies, rheumatoid factor, cryoglobulins, antinuclear antibodies), complicating the presentation. The heterogeneity of signs and symptoms has led to the development of multiple classification criteria. However, there is no accepted universal classification criterion for the diagnosis of Sjögren's syndrome. There are a limited number of studies that have been published on the epidemiology of Sjögren's syndrome, and the incidence and prevalence of the disease varies according to the classification criteria used. The data is further confounded by selection bias and misclassification bias, making it difficult for interpretation. The aim of this review is to understand the reported incidence and prevalence on pSS and sSS, the frequency of autoantibodies, and the risk of malignancy, which has been associated with pSS, taking into account the different classification criteria used. Keywords: Sjögren's syndrome, incidence, prevalence, classification criteria, autoantibodies, lymphomahttp://www.dovepress.com/the-epidemiology-of-sjoumlgrenrsquos-syndrome-peer-reviewed-article-CLEP |
| spellingShingle | Patel R Shahane A The epidemiology of Sjögren’s syndrome Clinical Epidemiology |
| title | The epidemiology of Sjögren’s syndrome |
| title_full | The epidemiology of Sjögren’s syndrome |
| title_fullStr | The epidemiology of Sjögren’s syndrome |
| title_full_unstemmed | The epidemiology of Sjögren’s syndrome |
| title_short | The epidemiology of Sjögren’s syndrome |
| title_sort | epidemiology of sj ouml gren rsquo s syndrome |
| url | http://www.dovepress.com/the-epidemiology-of-sjoumlgrenrsquos-syndrome-peer-reviewed-article-CLEP |
| work_keys_str_mv | AT patelr theepidemiologyofsjoumlgrenrsquossyndrome AT shahanea theepidemiologyofsjoumlgrenrsquossyndrome AT patelr epidemiologyofsjoumlgrenrsquossyndrome AT shahanea epidemiologyofsjoumlgrenrsquossyndrome |