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J Hematol Oncol. 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.

Author information

1
Autoimmune Diseases Unit, Department of Internal Medicine, Hospital CIMA-Sanitas, Barcelona, Spain.
2
Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
3
Transversal group for research in primary care, IDIBAPS, Consorci d'Atenció Primària de Salut Barcelona Esquerre (CAPSBE), Barcelona, Spain.
4
Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.
5
Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain.
6
Department of Internal Medicine, Complejo Hospitalario Universitario, Vigo, Spain.
7
Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca, Spain.
8
Department of Internal Medicine, Hospital Virgen de las Nieves, Granada, Spain.
9
Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Spain.
10
Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain.
11
Department of Internal Medicine, Hospital Infanta Sofía, Madrid, Spain.
12
Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Spain.
13
Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain.
14
Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain.
15
Department of Internal Medicine, Hospital Esperit Sant, Santa Coloma de Gramenet, Spain.
16
Department of Internal Medicine, Hospital de Fuenlabrada, Fuenlabrada, Spain.
17
Department of Internal Medicine, Hospital Virgen del Camino, Pamplona, Spain.
18
Department of Internal Medicine, Complejo Hospitalario Ruber Juan Bravo, Madrid, Spain.
19
Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Spain.
20
Department of Internal Medicine, Hospital de Salamanca, Salamanca, Spain.
21
Department of Internal Medicine, Hospital de Sagunto, Valencia, Spain.
22
Department of Hematology, ICMHO, Hospital Clinic, Barcelona, Spain.
23
Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain. mramos@clinic.cat.
24
Department of Medicine, University of Barcelona, Barcelona, Spain. mramos@clinic.cat.

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

PMID:
28416003
PMCID:
PMC5392920
DOI:
10.1186/s13045-017-0464-5
[Indexed for MEDLINE]
Free PMC Article

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