1. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome
- Author
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Brito-Zerón, Pilar, Kostov, Belchin, Fraile, Guadalupe, Caravia-Durán, Daniel, Maure, Brenda, Rascón, Francisco-Javier, Zamora, Mónica, Casanovas, Arnau, Lopez-Dupla, Miguel, Ripoll, Mar, Pinilla, Blanca, Fonseca, Eva, Akasbi, Miriam, de la Red, Gloria, Duarte-Millán, Miguel-Angel, Fanlo, Patricia, Guisado-Vasco, Pablo, Pérez-Alvarez, Roberto, Chamorro, Antonio J, Morcillo, César, Jiménez-Heredia, Iratxe, Sánchez-Berná, Isabel, López-Guillermo, Armando, Ramos-Casals, Manuel, SS Study Group GEAS-SEMI, The SS Study Group GEAS-SEMI, [Morcillo,C, Brito-Zerón,P] Autoimmune Diseases Unit, Department of Internal Medicine, Hospital CIMA-Sanitas, Barcelona, Spain. [Brito-Zerón, P: Sánchez-Berná,I, Ramos-Casals,M] Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain. [Kostov,B] Transversal group for research in primary care, IDIBAPS, Consorci d’Atenció Primària de Salut Barcelona Esquerre (CAPSBE), Barcelona, Spain. [Fraile,G] Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain. [Caravia-Durán,D] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain. [Maure,B] Department of Internal Medicine, Complejo Hospitalario Universitario, Vigo, Spain. [Rascón,FJ] Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca, Spain. [Zamora,M, and Sánchez-Berná,I] Department of Internal Medicine, Hospital Virgen de las Nieves, Granada, Spain. [Casanovas,A] Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Spain. [ Lopez-Dupla,M] Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain. [Ripoll,M] Department of Internal Medicine, Hospital Infanta Sofía, Madrid, Spain. [Pinilla,B] Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Spain. [Fonseca,E] Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain. [Akasbi,M] Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain. [de la Red,G] Department of Internal Medicine, Hospital Esperit Sant, Santa Coloma de Gramenet, Spain. [Duarte-Millán,MA] Department of Internal Medicine, Hospital de Fuenlabrada, Fuenlabrada, Spain. [Fanlo,P] Department of Internal Medicine, Hospital Virgen del Camino, Pamplona, Spain. [Guisado-Vasco,P] Department of Internal Medicine, Complejo Hospitalario Ruber Juan Bravo, Madrid, Spain. [Pérez-Alvarez,R] Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Spain. [Chamorro,AJ] Department of Internal Medicine, Hospital de Salamanca, Salamanca, Spain. [ Jiménez-Heredia,i] Department of Internal Medicine, Hospital de Sagunto, Valencia, Spain. [López-Guillermo,A] Department of Hematology, ICMHO, Hospital Clinic, Barcelona, Spain. [Ramos-Casals,M] Department of Medicine, University of Barcelona, Barcelona, Spain.
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Oncology ,Male ,Cancer Research ,Pathology ,Lymphoma ,Databases, Factual ,modelos de riesgos proporcionales ,humanos ,Diseases::Neoplasms::Neoplasms by Site::Endocrine Gland Neoplasms::Thyroid Neoplasms [Medical Subject Headings] ,Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings] ,Cohort Studies ,Enfermedad de hodgkin ,0302 clinical medicine ,immune system diseases ,hemic and lymphatic diseases ,Neoplasms ,Análisis de regresión ,evaluación de riesgos ,Gammopatía monoclonal de relevancia indeterminada ,Diseases::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Plasma Cell::Multiple Myeloma [Medical Subject Headings] ,Sjogren syndrome ,Stomach cancer ,estudios de cohortes ,Thyroid cancer ,Neoplasias de la tiroides ,Multiple myeloma ,mediana edad ,Síndrome de sjögren ,Cancer ,Labio ,neoplasias ,anciano ,Hematology ,Incidence ,Pronóstico ,Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma::Hodgkin Disease [Medical Subject Headings] ,Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Protein Disorders::Hypergammaglobulinemia::Monoclonal Gammopathy of Undetermined Significance [Medical Subject Headings] ,lcsh:Diseases of the blood and blood-forming organs ,adulto ,Middle Aged ,lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,Prognosis ,humanities ,pronóstico ,Sjogren's Syndrome ,030220 oncology & carcinogenesis ,Hematologic Neoplasms ,Linfoma de células b de la zona marginal ,Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Paraproteins::Cryoglobulins [Medical Subject Headings] ,Female ,Estudios de seguimiento ,Incidencia ,Anatomy::Stomatognathic System::Mouth::Lip [Medical Subject Headings] ,Adult ,medicine.medical_specialty ,Neoplasias gástricas ,Lymphoma, B-Cell ,lcsh:RC254-282 ,Risk Assessment ,incidencia ,03 medical and health sciences ,Crioglobulinas ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Incidence [Medical Subject Headings] ,Internal medicine ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies::Follow-Up Studies [Medical Subject Headings] ,medicine ,Diseases::Immune System Diseases::Autoimmune Diseases::Arthritis, Rheumatoid::Sjogren's Syndrome [Medical Subject Headings] ,Humans ,linfoma ,Molecular Biology ,Aged ,Proportional Hazards Models ,030203 arthritis & rheumatology ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Regression Analysis [Medical Subject Headings] ,Proportional hazards model ,business.industry ,lcsh:RC633-647.5 ,Diseases::Neoplasms::Neoplasms by Site::Digestive System Neoplasms::Gastrointestinal Neoplasms::Stomach Neoplasms [Medical Subject Headings] ,Research ,Mieloma múltiple ,medicine.disease ,neoplasias hematológicas ,Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma::Lymphoma, Non-Hodgkin::Lymphoma, B-Cell::Lymphoma, B-Cell, Marginal Zone [Medical Subject Headings] ,Sjögren syndrome ,stomatognathic diseases ,Risk Estimate ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis [Medical Subject Headings] ,business - 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)., This work is supported by the Grant Fondo de Investigaciones Sanitarias (MRC, INT15/00085) and by the CERCA Programme/Generalitat de Catalunya.
- Published
- 2017