Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Apr 17;10(1):90.
doi: 10.1186/s13045-017-0464-5.

Characterization and risk estimate of cancer in patients with primary Sjögren syndrome

Pilar Brito-Zerón  1   2 , Belchin Kostov  3 , Guadalupe Fraile  4 , Daniel Caravia-Durán  5 , Brenda Maure  6 , Francisco-Javier Rascón  7 , Mónica Zamora  8 , Arnau Casanovas  9 , Miguel Lopez-Dupla  10 , Mar Ripoll  11 , Blanca Pinilla  12 , Eva Fonseca  13 , Miriam Akasbi  14 , Gloria de la Red  15 , Miguel-Angel Duarte-Millán  16 , Patricia Fanlo  17 , Pablo Guisado-Vasco  18 , Roberto Pérez-Alvarez  19 , Antonio J Chamorro  20 , César Morcillo  1 , Iratxe Jiménez-Heredia  21 , Isabel Sánchez-Berná  2   8 , Armando López-Guillermo  22 , Manuel Ramos-Casals  23   24 ; SS Study Group GEAS-SEMI
Collaborators, Affiliations

Characterization and risk estimate of cancer in patients with primary Sjögren syndrome

Pilar Brito-Zerón et al. J Hematol Oncol. .

Abstract

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).

Keywords: Cancer; Lymphoma; Sjögren syndrome.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Survival curve for the development of solid and hematological cancer
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X. Primary Sjogren syndrome. BMJ. 2012;344:e3821. doi: 10.1136/bmj.e3821. - DOI - PubMed
    1. Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med. 1978;89:888–92. doi: 10.7326/0003-4819-89-6-888. - DOI - PubMed
    1. Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med. 2005;165:2337–44. doi: 10.1001/archinte.165.20.2337. - DOI - PubMed
    1. Papageorgiou A, Voulgarelis M, Tzioufas AG. Clinical picture, outcome and predictive factors of lymphoma in Sjӧgren syndrome. Autoimmun Rev. 2015;14:641–9. doi: 10.1016/j.autrev.2015.03.004. - DOI - PubMed
    1. Ramos-Casals M, De Vita S, Tzioufas AG. Hepatitis C virus, Sjögren’s syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer. Autoimmun Rev. 2005;4:8–15. doi: 10.1016/j.autrev.2004.04.004. - DOI - PubMed

Publication types