Identifier to cite or link to this item: http://hdl.handle.net/20.500.13003/12205
Characterization and risk estimate of cancer in patients with primary Sjogren syndrome
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eISSN: 1756-8722
WOS ID: 000399956800002
Scopus EID: 2-s2.0-85018384880
PMID: 28416003
Embase PUI: L615956540
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Brito-Zeron, Pilar; Kostov, Belchin; Fraile, Guadalupe; Caravia-Duran, Daniel; Maure, Brenda; Rascon, Francisco-Javier; Zamora, Monica; Casanovas, Arnau; Lopez-Dupla, Miguel; Ripoll, Mar; Pinilla, Blanca; Fonseca, Eva; Akasbi, Miriam; de la Red, Gloria; Duarte-Millan, Miguel-Angel; Fanlo, Patricia; Guisado-Vasco, Pablo; Perez-Alvarez, Roberto; Chamorro, Antonio J.; Morcillo, Cesar; Jimenez-Heredia, Iratxe; Sanchez-Berna, Isabel; Lopez-Guillermo, Armando; Ramos-Casals, Manuel; SS Study Grp GEAS-SEMIPublication date
2017-04-17Document type
research articleCitation
Brito-Zeron P, Kostov B, Fraile G, Caravia-Duran D, Maure B, Rascon FJ, et al. Characterization and risk estimate of cancer in patients with primary Sjogren syndrome. J Hematol Oncol. 2017 Apr 17;10:90.Abstract
Background: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjogren syndrome (SjS). Methods: We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender. The sex-and age-specific standardized incidence ratios (SIR) of cancer were estimated from 2012 Spanish mortality data. Results: After a mean follow-up of 91 months, 127 (9.8%) patients developed 133 cancers. The most frequent type of cancer was B-cell lymphoma (including 27 MALT and 19 non-MALT B-cell lymphomas). Systemic activity at diagnosis of primary SjS correlated with the risk of hematological neoplasia and cryoglobulins with a high risk of either B-cell or non-B-cell lymphoma subtypes. Patients with cytopenias had a high risk of non-MALT B-cell and non-B-cell cancer, while those with low C3 levels had a high risk of MALT lymphomas and those with monoclonal gammopathy and low C4 levels had a high risk of non-MALT lymphomas. The estimated SIR for solid cancer was 1. 13 and 11.02 for hematological cancer. SIRs for specific cancers were 36.17 for multiple myeloma and immunoproliferative diseases, 19.41 for Hodgkin lymphoma, 6.04 for other non-Hodgkin lymphomas, 5.17 for thyroid cancer, 4.81 for cancers of the lip and oral cavity, and 2.53 for stomach cancer. Conclusions: One third of cancers developed by patients with primary SjS are B-cell lymphomas. The prognostic factors identified at SjS diagnosis differed according to the subtype of B-cell lymphoma developed. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach).
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https://dx.doi.org/10.1186/s13045-017-0464-5Keywords
Sjogren syndromeCancer
Lymphoma
MeSH
Lymphoma, B-CellAged
Adult
Risk Assessment
Humans
Middle Aged
Neoplasms
Prognosis
Male
Databases, Factual
Female
Proportional Hazards Models
Cohort Studies
Hematologic Neoplasms
Incidence
DeCS
Estudios de CohortesNeoplasias Hematológicas
Incidencia
Modelos de Riesgos Proporcionales
Bases de Datos Factuales
Femenino
Masculino
Humanos
Persona de Mediana Edad
Neoplasias
Pronóstico
Anciano
Medición de Riesgo
Linfoma de Células B
Adulto