Brito-Zerón, PilarKostov, BelchinFraile, GuadalupeCaravia-Durán, DanielMaure, BrendaRascón, Francisco-JavierZamora, MónicaCasanovas, ArnauLopez-Dupla, MiguelRipoll, MarPinilla, BlancaFonseca, EvaAkasbi, Miriamde la Red, GloriaDuarte-Millán, Miguel-AngelFanlo, PatriciaGuisado-Vasco, PabloPérez-Alvarez, RobertoChamorro, Antonio JMorcillo, CésarJiménez-Heredia, IratxeSánchez-Berná, IsabelLópez-Guillermo, ArmandoRamos-Casals, Manuel2017-05-082017-05-082017-04-17Brito-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.http://hdl.handle.net/10668/2638The 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’Investigacions Biomèdiques 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 Hospitalario Universitario, 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 Internal Medicine, 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).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).enSjögren syndromeCancerLymphomaCrioglobulinasEstudios de seguimientoEnfermedad de hodgkinIncidenciaLabioLinfoma de células b de la zona marginalGammopatía monoclonal de relevancia indeterminadaMieloma múltiplePronósticoAnálisis de regresiónSíndrome de sjögrenNeoplasias gástricasNeoplasias de la tiroidesMedical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Paraproteins::CryoglobulinsMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies::Follow-Up StudiesMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma::Hodgkin DiseaseMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::IncidenceMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Anatomy::Stomatognathic System::Mouth::LipMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma::Lymphoma, Non-Hodgkin::Lymphoma, B-Cell::Lymphoma, B-Cell, Marginal ZoneMedical Subject Headings::Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Protein Disorders::Hypergammaglobulinemia::Monoclonal Gammopathy of Undetermined SignificanceMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Plasma Cell::Multiple MyelomaMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::PrognosisMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Regression AnalysisMedical Subject Headings::Diseases::Immune System Diseases::Autoimmune Diseases::Arthritis, Rheumatoid::Sjogren's SyndromeMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Site::Digestive System Neoplasms::Gastrointestinal Neoplasms::Stomach NeoplasmsMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Site::Endocrine Gland Neoplasms::Thyroid Neoplasmsresearch article28416003open access10.1186/s13045-017-0464-51756-8722PMC5392920