Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sjögren syndrome: analysis of 515 patients

Retamozo, Soledad; Gheitasi, H.; Quartuccio, Luca; Kostov, Belchin; Corazza, L.; Bové, Albert; Sisó‐Almirall, Antoni; Gandía, Myriam; Ramos‐Casals, Manuel; Vita, Salvatore De; Brito-Zerón, Pilar

doi:10.1093/rheumatology/kew194

Cited by 61 publications

(52 citation statements)

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“…Recent studies have confirmed severe parotid involvement, purpura, leukopenia, anti-La antibodies, raised levels of BAFF and beta2-microglobulin, cryoglobulins, monoclonal band, and hypocomplementemia as risk factors for B-cell lymphoma [24, 25, 29, 30]. The present study is the first to report that the prognostic role of these risk factors may differ according to the subtype of hematological cancer.…”

Section: Discussionsupporting

confidence: 61%

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: Discussionsupporting

confidence: 61%

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

Brito-Zerón¹,

et al. 2017

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“…Previous studies have shown that subgroups of pSS patients have a decreased survival, especially patients with high systemic disease activity 55 , and notably patients who develop severe systemic involvement such as cryoglobulinemic vasculitis 56 or who develop lymphoma 57 . However, those severe presentations are rare among patient with incident pSS, and do not affect the overall survival.…”

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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“…Serological or laboratory measures under consideration as potential candidate biomarkers associated with emergence of lymphoma in salivary glands among patients with SS included the following: presence of anemia, anti‐SSA and/or anti‐SSB positivity, cryoglobulinemia, high levels of Fms‐like tyrosine kinase 3 ligand, hypergammaglobulinemia, leukopenia, hypocomplementemia, that is, low C3 levels and/or low C4 levels, and monoclonal gammopathy (Table ). Hypocomplementemia and/or cryoglobulinemia were found to be predictive of lymphoma development in all relevant studies (Brito‐Zerón et al, ; De Vita et al, ; Kimman, Bossuyt, & Blockmans, ; Martel et al, ; Quartuccio et al, ; Ramos‐Casals et al, ; Retamozo et al, ; Tzioufas, Boumba, Skopouli, & Moutsopoulos, ).…”

Section: Resultsmentioning

confidence: 92%

World Workshop on Oral Medicine VII: Biomarkers predicting lymphoma in the salivary glands of patients with Sjögren’s syndrome—A systematic review

et al. 2019

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Objective To conduct a systematic review of studies exploring potential biomarkers for development, course, and efficacy of treatment of lymphomas in salivary glands of patients with Sjögren's syndrome. Material and Methods Eligible studies were identified through a comprehensive search of two databases, that is, PubMed and EMBASE. Quality of included articles was assessed with the “Quality In Prognosis Studies” (QUIPS) tool. The “CHecklist for critical Appraisal and data extraction for systematic Reviews of prediction Modelling Studies” (CHARMS) was used to facilitate data extraction. Results Fifty‐eight studies met the inclusion criteria. Only one study assessed the progression of lymphoma. Moderate risk of bias was detected in “outcome measurement,” “study participation,” and “study confounding” domains. Parotid gland enlargement, mixed monoclonal cryoglobulins, and low C4 levels represented strongest predictors of lymphoma development. The role of histological biomarkers, and specifically germinal centers, remains controversial. Clinical and methodological heterogeneity across studies precluded conduct of a meta‐analysis. Conclusions Specific biomarkers in combination with clinical manifestations represent potential candidates for advancing precision medicine approaches to lymphoma prediction in patients with Sjögren's syndrome. Current focus has increasingly been on genetic and epigenetic markers as candidate predictors. Predictive accuracy of key biomarker candidates remains to be tested in well‐designed prospectively followed Sjögren's syndrome cohorts.

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Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sjögren syndrome: analysis of 515 patients

Abstract: Patients with primary SS who fulfilled criteria for cryoglobulinaemic vasculitis at diagnosis are at higher risk of death.

Cited by 61 publications

References 25 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

World Workshop on Oral Medicine VII: Biomarkers predicting lymphoma in the salivary glands of patients with Sjögren’s syndrome—A systematic review

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