Helen L. Leavis
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View article: Proteins linked to type II interferon response in Sjögren’s disease: novel indicators for disease monitoring and predicting treatment response to leflunomide and hydroxychloroquine combination therapy
Proteins linked to type II interferon response in Sjögren’s disease: novel indicators for disease monitoring and predicting treatment response to leflunomide and hydroxychloroquine combination therapy Open
Objective To identify biomarkers and endotypes predictive of treatment response, monitor disease activity, and explore pathways associated with the clinical efficacy of leflunomide and hydroxychloroquine combination therapy (LEF/HCQ) in pa…
View article: Correction to: Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis
Correction to: Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis Open
View article: CVID Enteropathy Is Difficult To Treat and Shows a Heterogeneous Histopathology
CVID Enteropathy Is Difficult To Treat and Shows a Heterogeneous Histopathology Open
View article: Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis
Self-reported Clinical Outcomes and Quality of Life in Agammaglobulinemia: the Importance of an Early Diagnosis Open
Purpose Patients with (X-linked) agammaglobulinemia (XLA) suffer from severe, recurrent infections potentially leading to life-threatening complications such as sepsis, meningoencephalitis and chronic lung disease. Early diagnosis and time…
View article: Nasopharyngeal microbiome composition by SARS-CoV-2 presence and severity
Nasopharyngeal microbiome composition by SARS-CoV-2 presence and severity Open
The influence of SARS-CoV-2 on the nasopharyngeal microbiome, or vice-versa, is unclear. Nasopharyngeal swabs from Dutch healthcare workers (N = 257) and hospital outpatients with respiratory symptoms (N = 143), leftover after SARS-CoV-2 t…
View article: LB0005 REPURPSS-II: VALIDATION OF LEFLUNOMIDE-HYDROXYCHLOROQUINE COMBINATION THERAPY IN PATIENTS WITH PRIMARY SJÖGREN'S DISEASE: A DOUBLE-BLINDED, PLACEBO-CONTROLLED, RANDOMIZED TRIAL
LB0005 REPURPSS-II: VALIDATION OF LEFLUNOMIDE-HYDROXYCHLOROQUINE COMBINATION THERAPY IN PATIENTS WITH PRIMARY SJÖGREN'S DISEASE: A DOUBLE-BLINDED, PLACEBO-CONTROLLED, RANDOMIZED TRIAL Open
View article: Tocilizumab, sarilumab and anakinra in critically ill patients with COVID-19: a randomised, controlled, open-label, adaptive platform trial
Tocilizumab, sarilumab and anakinra in critically ill patients with COVID-19: a randomised, controlled, open-label, adaptive platform trial Open
Introduction Tocilizumab improves outcomes in critically ill patients with COVID-19. Whether other immune-modulator strategies are equally effective or better is unknown. Methods We investigated treatment with tocilizumab, sarilumab, anaki…
View article: Disseminated histoplasmosis induced hemophagocytic lymphohistiocytosis in an immunocompromised patient
Disseminated histoplasmosis induced hemophagocytic lymphohistiocytosis in an immunocompromised patient Open
Background: The search for the trigger of hemophagocytic lymphohistiocytosis (HLH) and its management can be challenging in immunocompromised patients. In HLH triggered by infection, immunosuppressive therapy is desired to suppress hyperin…
View article: Clara cell 16 kDa protein: an important marker for COVID-19 severity
Clara cell 16 kDa protein: an important marker for COVID-19 severity Open
The coronavirus disease 19 (COVID-19) is a disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) that invades lung epithelial cells and can lead to severe respiratory failure. In this study, we evaluated whethe…
View article: Gut microbial dysbiosis, IgA, and Enterococcus in common variable immunodeficiency with immune dysregulation
Gut microbial dysbiosis, IgA, and Enterococcus in common variable immunodeficiency with immune dysregulation Open
View article: Intravascular Large B‐Cell Lymphoma Presenting as Systemic Capillary Leak Syndrome With Immunological Phenomena: A Case Report
Intravascular Large B‐Cell Lymphoma Presenting as Systemic Capillary Leak Syndrome With Immunological Phenomena: A Case Report Open
Intravascular large B‐cell lymphoma (IVLBCL) has a high mortality rate, partly due to its heterogeneous presentation and rarity. We present a case of a 73‐year‐old woman who came into the emergency room in need of fluid resuscitation, inte…
View article: Targeting Common Immunopathological Pathways to Tackle the Divergent Clinical Presentation of Sjogren's Disease: One Strategy to Fit All?
Targeting Common Immunopathological Pathways to Tackle the Divergent Clinical Presentation of Sjogren's Disease: One Strategy to Fit All? Open
View article: Longitudinal assessment of immunoglobulin response and disease progression in critically ill patients with community acquired pneumonia
Longitudinal assessment of immunoglobulin response and disease progression in critically ill patients with community acquired pneumonia Open
In critically ill patients with CAP, IgG and IgM dynamics in the first week of ICU stay are not associated with clinically relevant changes in disease course, regardless of the causative pathogen.
View article: Paucity of gastrointestinal plasma cells in common variable immunodeficiency
Paucity of gastrointestinal plasma cells in common variable immunodeficiency Open
Purpose of review Common variable immunodeficiency enteropathy (CVID-E) is a noninfectious complication of CVID caused by chronic inflammation of the gastrointestinal (GI) tract. Based on literature, a paucity or lack of plasma cells, alth…
View article: The association of inflammatory markers with frailty and in-hospital mortality in older COVID-19 patients
The association of inflammatory markers with frailty and in-hospital mortality in older COVID-19 patients Open
View article: Association of autoantibodies with the IFN signature and NETosis in patients with systemic lupus erythematosus
Association of autoantibodies with the IFN signature and NETosis in patients with systemic lupus erythematosus Open
We identified a subgroup of patients with an IFN signature that express increased concentrations of antibodies against DNA and RNA-binding proteins, which can be useful for further patient stratification and a more targeted therapy. We did…
View article: Real-world experience with targeted therapy in patients with histiocytic neoplasms in the Netherlands and in Belgium
Real-world experience with targeted therapy in patients with histiocytic neoplasms in the Netherlands and in Belgium Open
View article: Pulmonary Computed Tomography Screening Frequency in Primary Antibody Deficiency
Pulmonary Computed Tomography Screening Frequency in Primary Antibody Deficiency Open
We identified risk factors that distinguished patients with PAD at risk for AD and ILD presence and progression, which could guide future screening frequency; however, independent and preferably prospective validation is needed.
View article: YajC, a predicted membrane protein, promotes <i>Enterococcus faecium</i> biofilm formation <i>in vitro</i> and in a rat endocarditis model
YajC, a predicted membrane protein, promotes <i>Enterococcus faecium</i> biofilm formation <i>in vitro</i> and in a rat endocarditis model Open
Biofilm formation is a critical step in the pathogenesis of difficult-to-treat Gram-positive bacterial infections. We identified that YajC, a conserved membrane protein in bacteria, plays a role in biofilm formation of the clinically relev…
View article: Clinical Validation of a Primary Antibody Deficiency Screening Algorithm for Primary Care
Clinical Validation of a Primary Antibody Deficiency Screening Algorithm for Primary Care Open
Purpose The diagnostic delay of primary antibody deficiencies (PADs) is associated with increased morbidity, mortality, and healthcare costs. Therefore, a screening algorithm was previously developed for the early detection of patients at …
View article: Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts
Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts Open
Objectives Adult-onset Still’s disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis; most doctors treating AOSD in the Netherlands treat <5 patients per year. Currently, there is no internationally …
View article: Neurological phenotype of adenosine deaminase 2 deficient patients: a cohort study
Neurological phenotype of adenosine deaminase 2 deficient patients: a cohort study Open
Background and purpose Patients with adenosine deaminase 2 (ADA2) deficiency can present with various neurological manifestations due to vasculopathies and autoinflammation. These include ischaemic and hemorrhagic stroke, but less clearly …
View article: The PID Odyssey 2030: outlooks, unmet needs, hurdles, and opportunities — proceedings from the IPOPI global multi-stakeholders’ summit (June 2022)
The PID Odyssey 2030: outlooks, unmet needs, hurdles, and opportunities — proceedings from the IPOPI global multi-stakeholders’ summit (June 2022) Open
IPOPI held its first Global Multi-Stakeholders’ Summit on 23-24 June 2022 in Cascais, Portugal. This IPOPI initiative was designed to set the stage for a stimulating forward-thinking meeting and brainstorming discussion among stakeholders …
View article: Leflunomide/hydroxychloroquine combination therapy targets type I IFN-associated proteins in patients with Sjögren’s syndrome that show potential to predict and monitor clinical response
Leflunomide/hydroxychloroquine combination therapy targets type I IFN-associated proteins in patients with Sjögren’s syndrome that show potential to predict and monitor clinical response Open
Objectives To assess to what extent leflunomide (LEF) and hydroxychloroquine (HCQ) therapy in patients with primary Sjögren’s syndrome (RepurpSS-I) targets type I IFN-associated responses and to study the potential of several interferon as…
View article: CCR9/CXCR5 Co-Expressing CD4 T Cells Are Increased in Primary Sjögren’s Syndrome and Are Enriched in PD-1/ICOS-Expressing Effector T Cells
CCR9/CXCR5 Co-Expressing CD4 T Cells Are Increased in Primary Sjögren’s Syndrome and Are Enriched in PD-1/ICOS-Expressing Effector T Cells Open
Primary Sjögren’s syndrome (pSS) is an autoimmune disease characterised by B cell hyperactivity. CXCR5+ follicular helper T cells (Tfh), CXCR5-PD-1hi peripheral helper T cells (Tph) and CCR9+ Tfh-like cells have been implicated in driving …
View article: Clinical Symptoms, Laboratory Parameters and Long-Term Follow-up in a National DADA2 Cohort
Clinical Symptoms, Laboratory Parameters and Long-Term Follow-up in a National DADA2 Cohort Open
Deficiency of adenosine deaminase-2 (DADA2) is an autosomal recessive autoinflammatory disease with an extremely variable disease presentation. This paper provides a comprehensive overview of the Dutch DADA2 cohort. We performed a retrospe…
View article: AB0117 ASSOCIATION OF AUTOANTIBODIES WITH THE IFN SIGNATURE AND NETOSIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS
AB0117 ASSOCIATION OF AUTOANTIBODIES WITH THE IFN SIGNATURE AND NETOSIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS Open
View article: POS0818 IN PRIMARY SJÖGREN'S SYNDROME CCR9/CXCR5 CO-EXPRESSING EFFECTOR CD4 T CELLS ARE INCREASED AND ARE ENRICHED IN PD-1/ICOS-EXPRESSING T CELLS
POS0818 IN PRIMARY SJÖGREN'S SYNDROME CCR9/CXCR5 CO-EXPRESSING EFFECTOR CD4 T CELLS ARE INCREASED AND ARE ENRICHED IN PD-1/ICOS-EXPRESSING T CELLS Open
View article: OP0215 IMMUNOME ANALYSIS INDICATES ROBUST INHIBITION OF PROINFLAMMATORY MEDIATORS UPON LEFLUNOMIDE-HYDROXYCHLOROQUINE COMBINATION THERAPY IN SJÖGREN'S SYNDROME: CXCR3 LIGANDS AS POTENTIAL INFLAMMATORY ENDOTYPE BIOMARKERS.
OP0215 IMMUNOME ANALYSIS INDICATES ROBUST INHIBITION OF PROINFLAMMATORY MEDIATORS UPON LEFLUNOMIDE-HYDROXYCHLOROQUINE COMBINATION THERAPY IN SJÖGREN'S SYNDROME: CXCR3 LIGANDS AS POTENTIAL INFLAMMATORY ENDOTYPE BIOMARKERS. Open
View article: Continuation of therapeutic dose heparin for critically ill patients with COVID-19
Continuation of therapeutic dose heparin for critically ill patients with COVID-19 Open