Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution

Quartuccio, Luca; Baldini, Chiara; Bartoloni, Elena; Priori, Roberta; Carubbi, Francesco; Corazza, L.; Alunno, Alessia; Colafrancesco, Serena; Luciano, N.; Giacomelli, Roberto; Gerli, Roberto; Valesini, Guido; Bombardieri, Stefano; Vita, Salvatore De

doi:10.1016/j.autrev.2015.07.002

Cited by 91 publications

(44 citation statements)

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“…However, this meta-analysis included cohorts of patients in whom the diagnosis of primary SjS was based on the 1993 criteria, whose fulfillment allows the inclusion of patients with negative Ro/La antibodies and negative biopsy. This creates a significant bias, since several studies have reported a lower risk of lymphoproliferation in immunonegative patients [8, 20]. Table 5 summarizes the studies based on the fulfillment of the 2002 criteria [8–11, 21–27].…”

Section: Discussionmentioning

confidence: 99%

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

Brito-Zerón¹,

et al. 2017

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BackgroundThe purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS).MethodsWe 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.ResultsAfter 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.ConclusionsOne 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).Electronic supplementary materialThe online version of this article (doi:10.1186/s13045-017-0464-5) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

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

Brito-Zerón¹,

et al. 2017

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“…Therefore, physicians may not consider it clinically useful to perform invasive diagnostic tests such as salivary gland biopsy if results of these tests would not affect clinical decisions regarding diagnosis and management. However, even if “seronegative” pSS patients probably have a lower risk of lymphoma development 50 , they have symptoms severe enough to require medical attention 51 . Besides its diagnostic value, salivary gland biopsy also gives important prognostic information, as patients with higher focus scores or ectopic germinal centers have a higher risk of systemic complications and lymphoma development 52–54 .…”

Section: Discussionmentioning

confidence: 99%

Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome

Gabriel

Crowson

Matteson

et al. 2017

Mayo Clinic Proceedings

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Objective To estimate the incidence and mortality rates of physician-diagnosed primary Sjögren’s syndrome (pSS) among residents of Olmsted County, Minnesota, and their evolution over time. Patients and Methods All medical records of patients with a diagnosis or suspicion of SS in Olmsted County, MN, from January 1, 2006 to December 31, 2015 were reviewed to identify incident cases of pSS (defined according to physician diagnosis). These cases were combined with a previous 1976–2005 incident cohort from the same population. Incidence rates were age and sex adjusted to the US white 2010 population. Survival rates were compared with the expected rates in the population of Minnesota. Results With 61 incident cases of pSS diagnosed in Olmsted County in 2006–2015, the total cohort included 172 patients with incident pSS in 1976–2015. Of the 172 patients, 151 (88%) were women and 161 (94%) were white, with a mean (SD) age at diagnosis of 58.3 (16.7) years. The average age- and sex- adjusted annual incidence for 2006–2015 was 5.9 per 100,000 population (95%CI 4.4–7.4), and overall incidence for the entire period was 5.8 (95% CI: 4.9–6.6) per 100,000. The incidence increased with calendar time over the 40-year period (P=.005). There was no difference in mortality in the pSS cohort compared to expected (standardized mortality ratio 1.15, 95%CI 0.86–1.50). Conclusions The average annual incidence of pSS in this population based-cohort was 5.8/100,000, with a progressive increase over the 40 years covered by the study. Overall survival of pSS patients was not different from the general population.

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“…Negative status for anti-Ro/SSA and/or anti-La/SSB is suggested to be a protective factor for evolution toward lymphoma in these patients [55]. However, the development of lymphomas in pSS is not confined only to serologically positive patients for anti-Ro and anti-La.…”

Section: Pathogenesismentioning

confidence: 99%

Epidemiology of Sjögren’s Syndrome—from an Oral Perspective

Bolstad

Skarstein

2016

Curr Oral Health Rep

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Oral symptoms are among the most distressing manifestations for patients with Sjögren’s syndrome (SS). The feeling of dry mouth is unpleasant, and hyposalivation may contribute to difficulty in speaking, chewing and swallowing and reduced quality of life. Reduced salivary flow increases the risk for dental caries and problems with prosthetic replacement. It seems that SS is not as frequently occurring as previously anticipated. Population-based prevalence studies on primary SS in Europe, conducted on large background populations and in accordance with the AECG criteria, reported of a prevalence of 1–9 cases per 10,000 people. This gives a combined prevalence of nearly 39/100,000 (~0.04 %). The cause of Sjögren’s syndrome is even now not fully understood, and the treatment of oral symptoms is still mostly palliative. Hopefully, useful information will appear from the new methods that are now available for genome wide association studies, epigenetics, DNA methylation studies, and proteomics. Similarly, this is anticipated for the immunological side of the story. The interferon signature, the interferon γ/interferon α mRNA ratio, and CXCL13 are among the proposed biomarkers of active disease. In this review, we provide an update on oral aspects of Sjögren’s syndrome with emphasis on the latest publications on these topics.

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Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution

Cited by 91 publications

References 24 publications

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

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

Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome

Epidemiology of Sjögren’s Syndrome—from an Oral Perspective

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