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dc.contributor.author
Chavez Flores, Alejandra Teresa
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Kostov, Belchin
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Solans Laqué, Roser
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Fraile, Guadalupe
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Maure, Brenda
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Feijoo-Massó, Carlos
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Rascon, Francisco Javier
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Perez Alvarez, Roberto
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Zamora, Mónica
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García-Pérez, Alicia
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Lopez-Dupla, Miguel
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Duarte Millán, Miguel Ángel
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Ripoll, Mar
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Fonseca, Eva
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Guisado, Pablo
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Pinilla,Blanca
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De la Red, Gloria
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Chamorro, Antonio J.
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Morcillo, César
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Fanlo, Patricia
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Soto-Cárdenas, María José
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Retamozo, Maria Soledad
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Ramos Casals, Manuel
dc.date.available
2020-10-26T19:17:46Z
dc.date.issued
2018-09
dc.identifier.citation
Chavez Flores, Alejandra Teresa; Kostov, Belchin; Solans Laqué, Roser; Fraile, Guadalupe; Maure, Brenda; et al.; Severe, life-threatening phenotype of primary Sjögren's syndrome: Clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry); Clinical & Exper Rheumatology; Clinical And Experimental Rheumatology; 36; 112; 9-2018; 121-129
dc.identifier.issn
0392-856X
dc.identifier.uri
http://hdl.handle.net/11336/116851
dc.description.abstract
To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren´s syndrome (SS).METHODS:The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain.RESULTS:Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reached to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved.CONCLUSIONS:13% of patients with primary SS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.
dc.format
application/pdf
dc.language.iso
eng
dc.publisher
Clinical & Exper Rheumatology
dc.rights
info:eu-repo/semantics/openAccess
dc.rights.uri
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.subject
Primary Sjögren’s syndrome
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Mortality
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Lymphoma
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Vasculitis
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Reumatología
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Medicina Clínica
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CIENCIAS MÉDICAS Y DE LA SALUD
dc.title
Severe, life-threatening phenotype of primary Sjögren's syndrome: Clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry)
dc.type
info:eu-repo/semantics/article
dc.type
info:ar-repo/semantics/artículo
dc.type
info:eu-repo/semantics/publishedVersion
dc.date.updated
2019-10-22T16:40:46Z
dc.identifier.eissn
1593-098X
dc.journal.volume
36
dc.journal.number
112
dc.journal.pagination
121-129
dc.journal.pais
Italia
dc.journal.ciudad
Pisa
dc.description.fil
Fil: Chavez Flores, Alejandra Teresa. Hospital Clinic Barcelona; España
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Fil: Kostov, Belchin. Gesclinic. Centre d'Assistència Primària ABS; España
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Fil: Solans Laqué, Roser. Hospital Vall d'Hebron; España
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Fil: Fraile, Guadalupe. Hospital Ramón y Cajal. Systemic Autoimmune Diseases Unit; España
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Fil: Maure, Brenda. Complejo Hospitalario Universitario, Vigo; España
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Fil: Feijoo-Massó, Carlos. Hospital Parc Taulí. Systemic Autoimmune Diseases Unit; España
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Fil: Rascon, Francisco Javier. Hospital Son Espases. Systemic Autoimmune Diseases Unit; España
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Fil: Perez Alvarez, Roberto. Hospital Do Meixoeiro. Department of Internal Medicine; España
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Fil: Zamora, Mónica. Hospital Virgen de las Nieves; España
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Fil: García-Pérez, Alicia. Hospital Universitario Central de Asturias; España
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Fil: Lopez-Dupla, Miguel. Hospital Joan XXIII; España
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Fil: Duarte Millán, Miguel Ángel. Hospital de Fuenlabrada; España
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Fil: Ripoll, Mar. Hospital Infanta Sofía; España
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Fil: Fonseca, Eva. Hospital de Cabueñes; España
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Fil: Guisado, Pablo. Complejo Hospitalario Ruber Juan Bravo. Department of Internal Medicine; España
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Fil: Pinilla,Blanca. Hospital Gregorio Marañón; España
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Fil: De la Red, Gloria. Hospital Esperit Sant Santa Coloma de Gramenet. Systemic Autoimmune Diseases Unit; España
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Fil: Chamorro, Antonio J.. Hospital Universitario de Salamanca. Systemic Autoimmune Diseases Unit; España
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Fil: Morcillo, César. Hospital CIMA-Sanitas. Systemic Autoimmune Diseases Unit; España
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Fil: Fanlo, Patricia. Hospital Virgen del Camino de Pamplona. Systemic Autoimmune Diseases Unit; España
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Fil: Soto-Cárdenas, María José. University of Cadiz. Department of Medicine; España
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Fil: Retamozo, Maria Soledad. Hospital Clinic. Department Of Autoimmune Diseases; España. 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
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Fil: Ramos Casals, Manuel. Hospital Clinic. Department Of Autoimmune Diseases; España
dc.journal.title
Clinical And Experimental Rheumatology
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/url/http://www.clinexprheumatol.org/article.asp?a=12884
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