Phenotyping Sjögren's syndrome: towards a personalised management of the disease

Autores
Brito Zeron, Pilar; Retamozo, Maria Soledad; Ramos-Casals Manuel
Año de publicación
2018
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Sjögren´s syndrome (SS) is a systemic autoimmune disease that mainly targets the exocrine glands. The disease overwhelmingly affects women around 30-60 years old, and more than 95% of patients present with oral and/or ocular dryness, although they may also develop a wide number of organ-specific systemic manifestations. The variable presentation is often linked to the influence of multiple personal determinants. In this review, we analyse the main geoepidemiological, immunological and histopathological determinants involved in the phenotypic expression of SS. With respect to sicca involvement, some patients (Asian, young-onset diagnosis, males and Ro-carriers) present with a less pronounced involvement in contrast to others with more pronounced dryness (seronegative, isolated La-carriers). With respect to the risk of developing systemic disease/poor outcomes, we propose a phenotypic-driven prognostic classification into patients at low risk (elderly-onset diagnosis, seronegative, isolated La-carriers), moderate risk (Black/African-american, young-onset diagnosis, Ro-carriers) and high risk (males, high focus score or presence of germinal centers in histopathological studies, RF-carriers, cryoglobulinaemic and hypocomplementaemic patients). Phenotype-based clustering of systemic autoimmune diseases may help physicians to offer a more personalised, cost-effective medical care of patients affected by these complex chronic diseases.
Fil: Brito Zeron, Pilar. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Hospital Cima-sanitas, Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España
Fil: Retamozo, Maria Soledad. Department Of Autoimmune Diseases, Hospital Clinic; España. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Sjögren Syndrome Research Group (agaur); España
Fil: Ramos-Casals Manuel. Universidad de Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España
Materia
Phenotyping
Sjögren's syndrome
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/92348
Acceder
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network_name_str CONICET Digital (CONICET)
spelling Phenotyping Sjögren's syndrome: towards a personalised management of the diseaseBrito Zeron, PilarRetamozo, Maria SoledadRamos-Casals ManuelPhenotypingSjögren's syndromehttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Sjögren´s syndrome (SS) is a systemic autoimmune disease that mainly targets the exocrine glands. The disease overwhelmingly affects women around 30-60 years old, and more than 95% of patients present with oral and/or ocular dryness, although they may also develop a wide number of organ-specific systemic manifestations. The variable presentation is often linked to the influence of multiple personal determinants. In this review, we analyse the main geoepidemiological, immunological and histopathological determinants involved in the phenotypic expression of SS. With respect to sicca involvement, some patients (Asian, young-onset diagnosis, males and Ro-carriers) present with a less pronounced involvement in contrast to others with more pronounced dryness (seronegative, isolated La-carriers). With respect to the risk of developing systemic disease/poor outcomes, we propose a phenotypic-driven prognostic classification into patients at low risk (elderly-onset diagnosis, seronegative, isolated La-carriers), moderate risk (Black/African-american, young-onset diagnosis, Ro-carriers) and high risk (males, high focus score or presence of germinal centers in histopathological studies, RF-carriers, cryoglobulinaemic and hypocomplementaemic patients). Phenotype-based clustering of systemic autoimmune diseases may help physicians to offer a more personalised, cost-effective medical care of patients affected by these complex chronic diseases.Fil: Brito Zeron, Pilar. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Hospital Cima-sanitas, Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); EspañaFil: Retamozo, Maria Soledad. Department Of Autoimmune Diseases, Hospital Clinic; España. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Sjögren Syndrome Research Group (agaur); EspañaFil: Ramos-Casals Manuel. Universidad de Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; EspañaClinical & Exper Rheumatology2018-09info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/92348Brito Zeron, Pilar; Retamozo, Maria Soledad; Ramos-Casals Manuel; Phenotyping Sjögren's syndrome: towards a personalised management of the disease; Clinical & Exper Rheumatology; Clinical And Experimental Rheumatology; 112; 3; 9-2018; 198-2090392-856XCONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://www.ncbi.nlm.nih.gov/pubmed/30156544info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:47:57Zoai:ri.conicet.gov.ar:11336/92348instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:47:57.855CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Phenotyping Sjögren's syndrome: towards a personalised management of the disease
title Phenotyping Sjögren's syndrome: towards a personalised management of the disease
spellingShingle Phenotyping Sjögren's syndrome: towards a personalised management of the disease
Brito Zeron, Pilar
Phenotyping
Sjögren's syndrome
title_short Phenotyping Sjögren's syndrome: towards a personalised management of the disease
title_full Phenotyping Sjögren's syndrome: towards a personalised management of the disease
title_fullStr Phenotyping Sjögren's syndrome: towards a personalised management of the disease
title_full_unstemmed Phenotyping Sjögren's syndrome: towards a personalised management of the disease
title_sort Phenotyping Sjögren's syndrome: towards a personalised management of the disease
dc.creator.none.fl_str_mv Brito Zeron, Pilar
Retamozo, Maria Soledad
Ramos-Casals Manuel
author Brito Zeron, Pilar
author_facet Brito Zeron, Pilar
Retamozo, Maria Soledad
Ramos-Casals Manuel
author_role author
author2 Retamozo, Maria Soledad
Ramos-Casals Manuel
author2_role author
author
dc.subject.none.fl_str_mv Phenotyping
Sjögren's syndrome
topic Phenotyping
Sjögren's syndrome
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Sjögren´s syndrome (SS) is a systemic autoimmune disease that mainly targets the exocrine glands. The disease overwhelmingly affects women around 30-60 years old, and more than 95% of patients present with oral and/or ocular dryness, although they may also develop a wide number of organ-specific systemic manifestations. The variable presentation is often linked to the influence of multiple personal determinants. In this review, we analyse the main geoepidemiological, immunological and histopathological determinants involved in the phenotypic expression of SS. With respect to sicca involvement, some patients (Asian, young-onset diagnosis, males and Ro-carriers) present with a less pronounced involvement in contrast to others with more pronounced dryness (seronegative, isolated La-carriers). With respect to the risk of developing systemic disease/poor outcomes, we propose a phenotypic-driven prognostic classification into patients at low risk (elderly-onset diagnosis, seronegative, isolated La-carriers), moderate risk (Black/African-american, young-onset diagnosis, Ro-carriers) and high risk (males, high focus score or presence of germinal centers in histopathological studies, RF-carriers, cryoglobulinaemic and hypocomplementaemic patients). Phenotype-based clustering of systemic autoimmune diseases may help physicians to offer a more personalised, cost-effective medical care of patients affected by these complex chronic diseases.
Fil: Brito Zeron, Pilar. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Hospital Cima-sanitas, Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España
Fil: Retamozo, Maria Soledad. Department Of Autoimmune Diseases, Hospital Clinic; España. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Sjögren Syndrome Research Group (agaur); España
Fil: Ramos-Casals Manuel. Universidad de Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España
description Sjögren´s syndrome (SS) is a systemic autoimmune disease that mainly targets the exocrine glands. The disease overwhelmingly affects women around 30-60 years old, and more than 95% of patients present with oral and/or ocular dryness, although they may also develop a wide number of organ-specific systemic manifestations. The variable presentation is often linked to the influence of multiple personal determinants. In this review, we analyse the main geoepidemiological, immunological and histopathological determinants involved in the phenotypic expression of SS. With respect to sicca involvement, some patients (Asian, young-onset diagnosis, males and Ro-carriers) present with a less pronounced involvement in contrast to others with more pronounced dryness (seronegative, isolated La-carriers). With respect to the risk of developing systemic disease/poor outcomes, we propose a phenotypic-driven prognostic classification into patients at low risk (elderly-onset diagnosis, seronegative, isolated La-carriers), moderate risk (Black/African-american, young-onset diagnosis, Ro-carriers) and high risk (males, high focus score or presence of germinal centers in histopathological studies, RF-carriers, cryoglobulinaemic and hypocomplementaemic patients). Phenotype-based clustering of systemic autoimmune diseases may help physicians to offer a more personalised, cost-effective medical care of patients affected by these complex chronic diseases.
publishDate 2018
dc.date.none.fl_str_mv 2018-09
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/92348
Brito Zeron, Pilar; Retamozo, Maria Soledad; Ramos-Casals Manuel; Phenotyping Sjögren's syndrome: towards a personalised management of the disease; Clinical & Exper Rheumatology; Clinical And Experimental Rheumatology; 112; 3; 9-2018; 198-209
0392-856X
CONICET Digital
CONICET
url http://hdl.handle.net/11336/92348
identifier_str_mv Brito Zeron, Pilar; Retamozo, Maria Soledad; Ramos-Casals Manuel; Phenotyping Sjögren's syndrome: towards a personalised management of the disease; Clinical & Exper Rheumatology; Clinical And Experimental Rheumatology; 112; 3; 9-2018; 198-209
0392-856X
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://www.ncbi.nlm.nih.gov/pubmed/30156544
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Clinical & Exper Rheumatology
publisher.none.fl_str_mv Clinical & Exper Rheumatology
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 13.13397

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