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Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations
Primary Sjögren’s syndrome (1° SS) is an autoimmune disorder characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth due to lymphocytic infiltrates of lacrimal and salivary glands. However, SS also affects many extraglandular systems. In SS patients, the pattern of extraglandular manife...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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2011
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7124115/ http://dx.doi.org/10.1007/978-1-60327-957-4_17 |
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author | Fox, Robert I. |
author_facet | Fox, Robert I. |
author_sort | Fox, Robert I. |
collection | PubMed |
description | Primary Sjögren’s syndrome (1° SS) is an autoimmune disorder characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth due to lymphocytic infiltrates of lacrimal and salivary glands. However, SS also affects many extraglandular systems. In SS patients, the pattern of extraglandular manifestations may have a close similarity with the vasculitic features seen in SLE patients that are mediated by immune complexes and complement. However, SS patients also have an increased frequency of lymphocytic infiltration into extraglandular tissues, as might be expected by their increased frequency of lymphoma in comparison to SLE patients. For example, SS patients need to be evaluated for interstitial nephritis (in contrast to the glomerulonephritis of SLE) or interstitial pneumonitis (in comparison to pleurisy of SLE). This chapter will focus on the clinical extraglandular manifestations of primary SS that are not specifically covered in other chapters. These extraglandular manifestations have led to a recently introduced “disease activity” and “organ damage index.” The recognition of these extraglandular manifestations is important since they have prognostic and therapeutic implications. The differential diagnosis of these extraglandular manifestations includes overlapping features with other autoimmune diseases (particularly systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, celiac sprue, and small- and medium-sized vessel vasculitis), infectious diseases that mimic autoimmune disease (particularly hepatitis C, HIV, syphilis, tuberculosis), and predisposition to drug toxicities that may involve extraglandular organs (particularly skin rashes, nephritis, pneumonitis, myositis, and hematopoietic abnormalities). |
format | Online Article Text |
id | pubmed-7124115 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
record_format | MEDLINE/PubMed |
spelling | pubmed-71241152020-04-06 Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations Fox, Robert I. Sjögren’s Syndrome Article Primary Sjögren’s syndrome (1° SS) is an autoimmune disorder characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth due to lymphocytic infiltrates of lacrimal and salivary glands. However, SS also affects many extraglandular systems. In SS patients, the pattern of extraglandular manifestations may have a close similarity with the vasculitic features seen in SLE patients that are mediated by immune complexes and complement. However, SS patients also have an increased frequency of lymphocytic infiltration into extraglandular tissues, as might be expected by their increased frequency of lymphoma in comparison to SLE patients. For example, SS patients need to be evaluated for interstitial nephritis (in contrast to the glomerulonephritis of SLE) or interstitial pneumonitis (in comparison to pleurisy of SLE). This chapter will focus on the clinical extraglandular manifestations of primary SS that are not specifically covered in other chapters. These extraglandular manifestations have led to a recently introduced “disease activity” and “organ damage index.” The recognition of these extraglandular manifestations is important since they have prognostic and therapeutic implications. The differential diagnosis of these extraglandular manifestations includes overlapping features with other autoimmune diseases (particularly systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, celiac sprue, and small- and medium-sized vessel vasculitis), infectious diseases that mimic autoimmune disease (particularly hepatitis C, HIV, syphilis, tuberculosis), and predisposition to drug toxicities that may involve extraglandular organs (particularly skin rashes, nephritis, pneumonitis, myositis, and hematopoietic abnormalities). 2011-04-12 /pmc/articles/PMC7124115/ http://dx.doi.org/10.1007/978-1-60327-957-4_17 Text en © Springer Science+Business Media, LLC 2011 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic. |
spellingShingle | Article Fox, Robert I. Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations |
title | Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations |
title_full | Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations |
title_fullStr | Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations |
title_full_unstemmed | Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations |
title_short | Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations |
title_sort | extraglandular manifestations of sjögren’s syndrome (ss): dermatologic, arthritic, endocrine, pulmonary, cardiovascular, gastroenterology, renal, urology, and gynecologic manifestations |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7124115/ http://dx.doi.org/10.1007/978-1-60327-957-4_17 |
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