Clinical features distinguishing lymphoma development in primary Sjögren's syndrome—A retrospective cohort study

Risselada, Arne; Kruize, Aike A.; Bijlsma, J. W. J.

doi:10.1016/j.semarthrit.2013.03.001

Cited by 50 publications

(31 citation statements)

References 37 publications

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“…On the other hand, at SS diagnosis, total ESSDAI score showed no significant differences between SS cases that latterly developed or not lymphoma. This finding is in agreement with a recent study by Risselada et al, where ESSDAI score could not clearly discriminate between patients with or without subsequent lymphoma development [45]. Ideally, measurement of ESSDAI in the year or 2 years before NHL diagnosis would be better to assess the potential predictive role of SS activity to lymphoma development.…”

Section: Discussionsupporting

confidence: 91%

Predicting the Outcome of Sjogren’s Syndrome-Associated Non-Hodgkin’s Lymphoma Patients

et al. 2015

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BackgroundNon-Hodgkin's lymphoma (NHL) development in Sjögren’s syndrome (SS) remains a potentially lethal complication and efforts should focus on the identification of predictors that could aid in appropriate therapeutic decisions.MethodsIn order to identify potential prognostic factors for outcome in SS-associated NHL, we retrospectively analyzed a cohort of 77 patients, diagnosed with NHL according to WHO classification criteria and meeting the American-European Consensus Classification (AECC) criteria for SS and examined the effect of SS-activity (defined as the EULAR SS disease activity index-ESSDAI) in the prognosis of SS-related NHLs, as defined in terms of overall and event-free survivals (OS and EFS). An event was defined as lymphoma relapse, treatment failure, disease progression, histological transformation or death. The effect of NHL clinical and laboratory characteristics was also investigated.ResultsMALT lymphomas constituted the majority (66.2%) of lymphomas. During the follow-up (median = 57.93 months), the 5-year OS was 90.91% (95% CI: 82.14–95.80%) and the EFS was 77.92% (95% CI: 67.37–85.82%). Patients with high ESSDAI score at lymphoma diagnosis had a greater risk for death (OR = 5.241, 95% CI: 1.034–26.568) or for event (OR = 4.317, 95% CI: 1.146–9.699, p = 0.008). These patients had also significantly worse EFS (HR = 4.541, 95% CI: 1.772–11.637) and OS (HR = 5.946, 95% CI: 1.259–28.077). In addition, post-chemotherapy ESSDAI improvement was significantly lower in patients who had experienced an event (p = 0.005). An unfavorable International prognostic index (IPI) score (high-intermediate/high) was associated with high risk of death and event (OR = 13.867, 95% CI: 2.656–72.387 and OR = 12.589, 95% CI: 3.911–40.526, respectively), worse EFS (log-rank p<0.001, HR = 8.718, 95% CI: 3.477–21.858), as well as with worse OS (log-rank p<0.001, HR = 11.414, 95% CI: 2.414–53.974). After adjustment for identified risk factors, IPI score retained a significant prognostic role following by a strong effect of ESSDAI in survival outcomes.ConclusionsAt the point of NHL diagnosis, IPI and ESSDAI might be proved useful predictive tools in SS-associated lymphoma prognosis, directing to a more patient-tailored approach.

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Section: Discussionsupporting

confidence: 91%

Predicting the Outcome of Sjogren’s Syndrome-Associated Non-Hodgkin’s Lymphoma Patients

et al. 2015

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“…In the same context, monoclonal gammopathy, [29,38,39] hypocomplementemia, and cryoglobulinemia [5,9,16,18,28,29,34,37,40] previously associated with malignant transformation, possibly as a result of excessive B-cell activation, have also been shown to be independently related to NHL occurrence and increased mortality. [5,40,41] Monoclonal mixed cryoprecipitates, reported as a detrimental prognostic factor for SS-related lymphomagenesis, [17] contain monoclonal RF, secreted by a subset of malignant B-cells derived by clonally expanded B cells exhibiting RF activity, [42] which has been emerged as an independent predictor for NHL in both Greek and French cohorts.…”

Section: Discussionmentioning

confidence: 99%

Predicting the risk for lymphoma development in Sjogren syndrome

2016

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“…Several patients were eventually lost to follow-up; the Netherlands do not have national registries to retrieve lost patient outcomes like lymphoma. Additionally, patients with EGM and evident SS are less likely to have a salivary gland biopsy performed, because it is deemed unnecessary for their diagnosis 10 11. This group of patients is therefore underrepresented in histology studies.…”

Section: Discussionmentioning

confidence: 99%

“…Of the 195 patients described in more detail elsewhere,10 11 174 patients had documented minor salivary gland histology and were included in this analysis. The retrospective and anonymous nature of this report made ethical approval unnecessary, following the ethics guidelines of our hospital.…”

Section: Methodsmentioning

confidence: 99%

“…For calculating ESSDAI/EGM scores, missing laboratory data were interpreted as being normal/absent if no value was available for any time point, the last value was carried forward, or the first value was carried backward. Since it can be questioned whether lymphoma and manifestations occurring during its course should be scored under pSS,11 scores of the cumulative ESSDAI and EGM are displayed with and without counting those manifestations most likely related to NHL. The diagnosis of NHL was made according to the WHO classification with staging following the Ann Arbor system.…”

Section: Methodsmentioning

confidence: 99%

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The prognostic value of routinely performed minor salivary gland assessments in primary Sjögren's syndrome

et al. 2014

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Clinical features distinguishing lymphoma development in primary Sjögren's syndrome—A retrospective cohort study

Cited by 50 publications

References 37 publications

Predicting the Outcome of Sjogren’s Syndrome-Associated Non-Hodgkin’s Lymphoma Patients

Predicting the Outcome of Sjogren’s Syndrome-Associated Non-Hodgkin’s Lymphoma Patients

Predicting the risk for lymphoma development in Sjogren syndrome

The prognostic value of routinely performed minor salivary gland assessments in primary Sjögren's syndrome

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