Attenuation of Follicular Helper T Cell-Dependent B Cell Hyperactivity by Abatacept Treatment in Primary Sjögren's Syndrome.

Objective: To assess the effect of abatacept (CTLA-4Ig), which limits T cell activation, on homeostasis of CD4+ T cell subsets and T cell-dependent B cell hyperactivity in patients with primary Sjögren's syndrome (SS).

Methods: Fifteen patients with primary SS treated with abatacept were included. Circulating CD4+ T cell and B cell subsets were analyzed by flow cytometry at baseline, during the treatment course, and after treatment was completed.

Detailed Analysis of the Articular Domain in Patients with Primary Sjögren Syndrome.

Objective: We used the 28-joint Disease Activity Score (DAS28) and the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI) articular domain to assess the effect of rituximab (RTX) and abatacept (ABA) on articular involvement in primary Sjögren syndrome (pSS).

Methods: Patients with pSS treated with RTX (n = 18) or ABA (n = 13) and having a DAS28 erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) level ≥ 3.2 at baseline were selected.

Physical fatigue characterises patient experience of primary Sjögren's syndrome.

Objectives: Besides ocular and oral dryness, fatigue is a major symptom in patients with primary Sjögren's syndrome (pSS). Our aim was to investigate the importance of fatigue in relation to other symptoms experienced as well as to evaluate the effect of rituximab treatment on fatigue in pSS patients with active disease.

Methods: This analysis was based on data from our open-label rituximab study in 28 pSS patients.

Abatacept treatment of patients with primary Sjögren's syndrome results in a decrease of germinal centres in salivary gland tissue.

Objectives: The aim of this study was to assess the histopathological changes in parotid gland tissue of primary Sjögren's syndrome (pSS) patients treated with abatacept.

Methods: In all 15 pSS patients included in the open-label Active Sjögren Abatacept Pilot (ASAP, 8 abatacept infusions) study parotid gland biopsies were taken before treatment and at 24 weeks of follow up. Biopsies were analysed for pSS-related histopathological features and placed in context of clini- cal responsiveness as assessed with EULAR Sjögren's syndrome disease activity index (ESSDAI).

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

Objective: To develop and validate ClinESSDAI (Clinical European League Against Rheumatism Sjögren's Syndrome Disease Activity Index), ie, ESSDAI without the biological domain.

Patients And Methods: The 702 fictive vignettes derived from 96 real cases of primary Sjögren's syndrome of the ESSDAI development study were used. As for ESSDAI development, the physician assessment of disease activity (0-10 scale) was used as the 'gold standard' in a multivariate model for weighting domains, after removing the biological domain.

Dental Implants in Patients with Sjögren's Syndrome.

Background: Limited evidence is available for applying dental implants in Sjögren's syndrome (SS) patients.

Purpose: This study aims to retrospectively assess clinical outcome of implant therapy in a cohort of well-classified patients with SS.

Materials And Methods: All SS patients attending the University Medical Center Groningen for follow-up (n = 406) were asked whether they had implants.

Development of the Sjögren's Syndrome Responder Index, a data-driven composite endpoint for assessing treatment efficacy.

Objectives: To determine which outcome measures detected rituximab efficacy in the Tolerance and Efficacy of Rituximab in Sjögren's Disease (TEARS) trial and to create a composite endpoint for future trials in primary SS (pSS).

Methods: Post hoc analysis of the multicentre randomized placebo-controlled double-blind TEARS trial. The results were validated using data from two other randomized controlled trials in pSS, assessing rituximab (single-centre trial in the Netherlands) and infliximab, respectively.

Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI).

Objectives: To define disease activity levels, minimal clinically important improvement (MCII) and patient-acceptable symptom state (PASS) with the primary Sjögren's syndrome (SS) disease activity indexes: European League Against Rheumatism (EULAR) SS disease activity index (ESSDAI) and EULAR SS patient-reported index (ESSPRI).

Methods: For 790 patients from two large prospective cohorts, ESSDAI, physician evaluation of disease activity, ESSPRI and patients' satisfaction with their current health status were recorded. Receiver operating characteristic curve analyses and anchoring methods were used to estimate disease activity levels of ESSDAI and the PASS of ESSPRI.

Treatment of primary Sjögren's syndrome with anti-CD20 therapy (rituximab). A feasible approach or just a starting point?

Introduction: In vitro and in vivo experimental data have suggested new immunopathogenic mechanisms in primary Sjögren's syndrome (pSS). The availability of targeted treatment modalities has opened new ways to selectively target these mechanistic pathways in vivo. Amongst these new treatment modalities, monoclonal antibodies specific for the B-cell surface molecule CD20 have been shown to be the most promising treatment option to date.

Health-related quality of life, employment and disability in patients with Sjogren's syndrome.

Objective: To compare health-related quality of life (HR-QOL), employment and disability of primary and secondary SS (pSS and sSS, respectively) patients with the general Dutch population.

Methods: HR-QOL, employment and disability were assessed in SS patients regularly attending the University Medical Center Groningen (n = 235). HR-QOL, employment and disability were evaluated with the Short Form-36 questionnaire (SF-36) and an employment and disability questionnaire.

Pneumococcal septicaemia with Purpura fulminans in an 11-month-old child.

Purpura fulminans (PF) is a syndrome characterised by acute onset of rapidly progressive haemorrhagic necrosis of the skin due to dermal vascular thrombosis, mainly occurring during meningococcal sepsis. It occurs rarely in the course of infection with Streptococcus pneumoniae and most cases report Meningococcus as the causing agent. This is a case report of successful conservative limb-preserving management of PF and sepsis caused by Streptococcus pneumoniae in an 11-month-old girl.