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Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use.

ABSTRACT: The heightened risk of non-Hodgkin lymphoma (NHL) development in primary Sjogren syndrome (SS) is well established. Several adverse clinical and laboratory predictors have been described. In the current work, we aimed to formulate a predictive score for NHL development, based on clinical, serological, and histopathological findings at the time of SS diagnosis. In the present case-control study of 381 primary SS patients and 92 primary SS patients with concomitant NHL, clinical, serological, and histopathological variables at the time of SS diagnosis were retrospectively recorded. For the identification of predictors for NHL development univariate and multivariate models were constructed. Salivary gland enlargement (SGE), lymphadenopathy, Raynaud phenomenon, anti-Ro/SSA or/and anti-La/SSB autoantibodies, rheumatoid factor (RF) positivity, monoclonal gammopathy, and C4 hypocomplementemia were shown to be independent predictors for NHL development. On the basis of the number of independent risk factors identified, a predictive risk score for NHL development was formulated. Thus, patients presenting with ?2 risk factors had a 3.8% probability of NHL development, those with 3 to 6 risk factors 39.9% (OR (95%CI): 16.6 [6.5-42.5], P?

SUBMITTER: Fragkioudaki S

PROVIDER: S-EPMC4998301 | biostudies-literature | 2016 Jun

REPOSITORIES: biostudies-literature

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Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use.

Fragkioudaki Sofia S Mavragani Clio P CP Moutsopoulos Haralampos M HM

Medicine 20160601 25

The heightened risk of non-Hodgkin lymphoma (NHL) development in primary Sjogren syndrome (SS) is well established. Several adverse clinical and laboratory predictors have been described. In the current work, we aimed to formulate a predictive score for NHL development, based on clinical, serological, and histopathological findings at the time of SS diagnosis. In the present case-control study of 381 primary SS patients and 92 primary SS patients with concomitant NHL, clinical, serological, and ...[more]

PMID: 27336863

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Project description:ObjectiveTo assess outcomes of repeat rituximab cycles and identify predictors of sustained clinical response in systemic manifestations of primary Sjögren syndrome (pSS).MethodsAn observational study was conducted in 40 rituximab-treated patients with pSS. Clinical response was defined as a 3-point or more reduction in the European League Against Rheumatism (EULAR) Sjögren Disease Activity Index (ESSDAI) at 6 months from baseline. Peripheral blood B cells were measured using highly sensitive flow cytometry. Predictors of sustained response (within two rituximab cycles) were analyzed using penalized logistic regression.ResultsThirty-eight out of 40 patients had moderate to severe systemic disease (ESSDAI >5). Main domains were articular (73%), mucocutaneous (23%), hematological (20%), and nervous system (18%). Twenty-eight out of 40 (70%) patients were on concomitant immunosuppressants. One hundred sixty-nine rituximab cycles were administered with a total follow-up of 165 patient-years. In cycle 1 (C1), 29/40 (73%) achieved ESSDAI response. Of C1 responders, 23/29 received retreatment on clinical relapse, and 15/23 (65%) responded. Of the 8/23 patients who lost response, these were due to secondary non-depletion and non-response (2NDNR; 4/23 [17%] as we previously observed in systemic lupus erythematosus with antirituximab antibodies, inefficacy = 2/23, and other side effects = 2/23). Within two cycles, 13/40 (33%) discontinued therapy. In multivariable analysis, concomitant immunosuppressant (odds ratio 7.16 [95% confidence interval: 1.37-37.35]) and achieving complete B-cell depletion (9.78 [1.32-72.25]) in C1 increased odds of response to rituximab. At 5 years, 57% of patients continued on rituximab.ConclusionOur data suggest that patients with pSS should be co-prescribed immunosuppressant with rituximab, and treatment should aim to achieve complete depletion. About one in six patients develop 2NDNR in repeat cycles. Humanized or type 2 anti-CD20 antibodies may improve clinical response in extra-glandular pSS.

Project description:BackgroundInterpersonal firearm violence among youth is a substantial public health problem, and emergency department (ED) physicians require a clinical screening tool to identify high-risk youth.ObjectiveTo derive a clinically feasible risk index for firearm violence.Design24-month prospective cohort study.SettingUrban, level 1 ED.ParticipantsSubstance-using youths, aged 14 to 24 years, seeking ED care for an assault-related injury and a proportionately sampled group of non-assault-injured youth enrolled from September 2009 through December 2011.MeasurementsFirearm violence (victimization/perpetration) and validated questionnaire items.ResultsA total of 599 youths were enrolled, and presence/absence of future firearm violence during follow-up could be ascertained in 483 (52.2% were positive). The sample was randomly split into training (75%) and post-score-construction validation (25%) sets. Using elastic-net penalized logistic regression, 118 baseline predictors were jointly analyzed; the most predictive variables fell predominantly into 4 domains: violence victimization, community exposure, peer influences, and fighting. By selection of 1 item from each domain, the 10-point SaFETy (Serious fighting, Friend weapon carrying, community Environment, and firearm Threats) score was derived. SaFETy was associated with firearm violence in the validation set (odds ratio [OR], 1.47 [95% CI, 1.23 to 1.79]); this association remained (OR, 1.44 [CI, 1.20 to 1.76]) after adjustment for reason for ED visit. In 5 risk strata observed in the training data, firearm violence rates in the validation set were 18.2% (2 of 11), 40.0% (18 of 45), 55.8% (24 of 43), 81.3% (13 of 16), and 100.0% (6 of 6), respectively.LimitationsThe study was conducted in a single ED and involved substance-using youths. SaFETy was not externally validated.ConclusionThe SaFETy score is a 4-item score based on clinically feasible questionnaire items and is associated with firearm violence. Although broader validation is required, SaFETy shows potential to guide resource allocation for prevention of firearm violence.Primary funding sourceNational Institute on Drug Abuse R01024646.

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Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use.

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Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use.

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