Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients

Brito-Zerón, Pilar; Kostov, Belchin; Solans, Roser; Fraile, G.; Suárez-Cuervo, Carlos; Casanovas, A.; Rascόn, F.-J.; Qanneta, Rami; Pérez-Álvarez, R.; Ripoll, M.; Akasbi, M.; Pinilla, Blanca; Bosch, J.; Nava-Mateos, J J; Díaz-Lόpez, B.; Morera-Morales, Maria Lluisa; Gheitasi, H.; Retamozo, Soledad; Ramos‐Casals, M.

doi:10.1136/annrheumdis-2014-206418

Cited by 161 publications

(113 citation statements)

References 28 publications

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“…However, systemic involvement has increasingly been recognized as a key part of the disease spectrum with a significant weight in dictating the prognosis and survival [13]. Among the systemic manifestations of SjS, lymphoma is one of the worst complications that physicians should expect.…”

Section: Discussionmentioning

confidence: 99%

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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%

“…Diagnostic tests for SjS (ocular tests, parotid scintigraphy, and salivary gland biopsy) were performed according to the European Community Study Group recommendations [12]. Clinical and laboratory data were collected and computerized according to a standard previously reported protocol [13]. …”

Section: Methodsmentioning

confidence: 99%

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

Brito-Zerón¹,

et al. 2017

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“…Compared to the general population, pSS patients have an increased mortality risk. The standardized mortality ratio (ratio of observed deaths in the study group to expected deaths in the general population, SMR) of pSS patients is on average 2.86, showing that pSS has an impact on patients' survival 96, 97. The leading cause of mortality in pSS is lymphoma with a lymphoma-specific SMR of 7.89, associating lymphoproliferative disorders directly with death in pSS 49.…”

Section: Prognosismentioning

confidence: 96%

Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment

Both¹,

Dalm²,

Hagen³

et al. 2017

Int. J. Med. Sci.

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Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. The pathogenesis of pSS is currently not well understood, but increased activation of B cells followed by immune complex formation and autoantibody production are thought to play important roles. pSS is diagnosed using the American-European consensus group (AECG) classification criteria which include subjective symptoms and objective tests such as histopathology and serology. The treatment of pSS warrants an organ based approach, for which local treatment (teardrops, moistures) and systemic therapy (including non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, disease-modifying antirheumatic drugs (DMARDS) and biologicals) can be considered. Biologicals used in the treatment of pSS mainly affect the total numbers of B cells (B cell depletion (Rituximab)) or target proteins required for B cell proliferation and/or activation (e.g. B cell activating factor (BAFF)) resulting in decreased B cell activity.The aim of this review is to provide physicians a general overview concerning the pathogenesis, diagnosis and management of pSS patients.

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“…This domain is the more frequently involved ESSDAI domain affecting one-third to two-thirds of the patients in recent cohorts 3 6 7. In the context of biological studies focusing on identification of new biomarkers, association with disease activity is a crucial question to be answered, since the purpose of these markers is to know if they are implicated in the disease process.…”

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confidence: 99%

Development of the ClinESSDAI: a clinical score without biological domain. A tool for biological studies

et al. 2016

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ClinESSDAI appears valid and very close to the original ESSDAI. This score provides an accurate evaluation of disease activity independent of B-cell biomarkers. It could be used in various circumstances: (i) in biological/clinical studies to avoid data collinearity, (ii) in clinical trials, as secondary endpoint, to detect change independent of biological effect of the drug, (iii) in clinical practice to assess disease activity for visits where immunological tests have not been done.

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Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients

Abstract: Patients with primary SS, who present at diagnosis with high systemic activity (ESSDAI ≥14) and/or predictive immunological markers (especially those with more than one), are at higher risk of death.

Cited by 161 publications

References 28 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

Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment

Development of the ClinESSDAI: a clinical score without biological domain. A tool for biological studies

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