RT Journal Article
T1 Characterization and risk estimate of cancer in patients with primary Sjögren syndrome
A1 Brito-Zerón, Pilar
A1 Kostov, Belchin
A1 Fraile, Guadalupe
A1 Caravia-Durán, Daniel
A1 Maure, Brenda
A1 Rascón, Francisco-Javier
A1 Zamora, Mónica
A1 Casanovas, Arnau
A1 Lopez-Dupla, Miguel
A1 Ripoll, Mar
A1 Pinilla, Blanca
A1 Fonseca, Eva
A1 Akasbi, Miriam
A1 de la Red, Gloria
A1 Duarte-Millán, Miguel-Angel
A1 Fanlo, Patricia
A1 Guisado-Vasco, Pablo
A1 Pérez-Alvarez, Roberto
A1 Chamorro, Antonio J
A1 Morcillo, César
A1 Jiménez-Heredia, Iratxe
A1 Sánchez-Berná, Isabel
A1 López-Guillermo, Armando
A1 Ramos-Casals, Manuel
K1 Sjögren syndrome
K1 Cancer
K1 Lymphoma
K1 Crioglobulinas
K1 Estudios de seguimiento
K1 Enfermedad de hodgkin
K1 Incidencia
K1 Labio
K1 Linfoma de células b de la zona marginal
K1 Gammopatía monoclonal de relevancia indeterminada
K1 Mieloma múltiple
K1 Pronóstico
K1 Análisis de regresión
K1 Síndrome de sjögren
K1 Neoplasias gástricas
K1 Neoplasias de la tiroides
AB BACKGROUND:The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren 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).
PB BioMed Central
YR 2017
FD 2017-04-17
LK http://hdl.handle.net/10668/2638
UL http://hdl.handle.net/10668/2638
LA en
NO Brito-Zerón P, Kostov B, Fraile G, Caravia-Durán D, Maure B, Rascón FJ et al. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome. J Hematol Oncol. 2017 Apr 17;10(1):90.
NO The members of the SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI) involved in this project have been:M. Ramos-Casals (Coordinator), P. Brito-Zerón, S. Retamozo, A. Bové, H. Gheitasi,I. Sánchez-Berná, J. Gratacós (Sjögren Syndrome Research Group-AGAUR, Laboratory of Autoimmune Diseases Josep Font, Institut d’InvestigacionsBiomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona); G. Fraile, J. Nava-Mateos (Department of Internal Medicine, Hospital Ramón y Cajal, Madrid); B.Díaz-López, D. Caravia-Duran, A. García-Pérez (Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo); A.Casanovas, M.L. Morera-Morales, T.E. Junco-Russeau (Department of Internal Medicine, Hospital Parc Taulí, Sabadell); F.J. Rascón, L. Pallarés (Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca); R. Pérez-Alvarez, M. Perez-de-Lis (Department of Internal Medicine, Hospital Alvaro Cunqueiro); M. Ripoll, C. Moreno-delaSantaGarcía (Department of Internal Medicine, Hospital Infanta Sofía, Madrid); B. Pinilla, C. López-GónzalezCobos (Department of Internal Medicine, Hospital Gregorio Marañón, Madrid); M. Akasbi, I. García-Sanchez (Department of Internal Medicine, Hospital Infanta Leonor, Madrid); B.Maure (Department of Internal Medicine, Complejo HospitalarioUniversitario, Vigo); E. Fonseca (Department of Internal Medicine, Hospital de Cabueñes, Gijón); M.A. Duarte-Millán, J. Canora (Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Madrid); G de la Red, N. Msabri (Department of Internal Medicine, Hospital Esperit Sant, Santa Coloma de Gramenet, Barcelona); A.J. Chamorro, S. Rodríguez Rodríguez (Department of Internal Medicine, Complejo Hospitalario de Salamanca, Salamanca); I. Jiménez-Heredia (Department of Internal Medicine, Hospital de Sagunt, Valencia, Spain); M.A. López-Dupla, J.A.Porras-Ledantes, B. Villar-Navas, J. Ramos-Rodriguez (Department of Internal Medicine, Hospital Joan XXIII, Tarragona); P. Brito-Zerón, C. Morcillo (Department of Internal Medicine, Hospital CIMA-Sanitas, Barcelona); M. Zamora, I. Sánchez-Berná (Department of Internal Medicine, Hospital Virgen de las Nieves, Granada); P. Fanlo (Department of InternalMedicine, Hospital Virgen del Camino, Pamplona); P. Guisado-Vasco (Department of Internal Medicine, Complejo Hospitalario Ruber Juan Bravo, Madrid); B. Kostov, A. Sisó-Almirall (Primary Care Research Group, IDIBAPS,Centre d’Assistència Primària ABS Les Corts, CAPSE, Barcelona, Spain).
NO This work is supported by the Grant Fondo de Investigaciones Sanitarias (MRC, INT15/00085) and by the “CERCA Programme/Generalitat de Catalunya”.
DS RISalud
RD Apr 19, 2025