Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA.

Incidence of giant cell arteritis in six districts of Paris, France (2015-2017).

This prospective population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris. GCA cases diagnosed between 2015 and 2017 were obtained from local hospital and community-based physicians and the national health insurance system database. Criteria for inclusion were living in the study area at that time and fulfilling the 1990 American College of Rheumatology classification criteria and/or its expanded version.

Identification of Functional and Expression Polymorphisms Associated With Risk for Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis.

Objective: To identify risk alleles relevant to the causal and biologic mechanisms of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods: A genome-wide association study and subsequent replication study were conducted in a total cohort of 1,986 cases of AAV (patients with granulomatosis with polyangiitis [Wegener's] [GPA] or microscopic polyangiitis [MPA]) and 4,723 healthy controls. Meta-analysis of these data sets and functional annotation of identified risk loci were performed, and candidate disease variants with unknown functional effects were investigated for their impact on gene expression and/or protein function.

Do vaccinations affect the clinical course of systemic necrotising vasculitis? A prospective observational web-based study.

Objectives: To estimate the impact of vaccinations, infections and traumatic life events on the disease activity of a web-based cohort of systemic necrotising vasculitis (SNV) patients.

Methods: Adults diagnosed with SNV self-reported vaccinations, infectious episodes and traumatic life events every 3 months during follow-up on a secure dedicated website. Participants reported information on disease activity assessed with 3 scores: the French version of the Medical Outcome Study Short Form-36 (SF-36), the visual numerical scale for Patient Global Assessment (PGA) and the modified Disease Extent Index (mDEI).

Rituximab: Recommendations of the French Vasculitis Study Group (FVSG) for induction and maintenance treatments of adult, antineutrophil cytoplasm antibody-associated necrotizing vasculitides.

Increasing rituximab prescription for ANCA-associated necrotizing vasculitides justifies the publication of recommendations for clinicians. Rituximab is approved in the United States to induce and maintain remission. In Europe, rituximab was recently approved for remission induction.

Association of granulomatosis with polyangiitis (Wegener's) with HLA-DPB1*04 and SEMA6A gene variants: evidence from genome-wide analysis.

Objective: To identify genetic determinants of granulomatosis with polyangiitis (Wegener's) (GPA).

Methods: We carried out a genome-wide association study (GWAS) of 492 GPA cases and 1,506 healthy controls (white subjects of European descent), followed by replication analysis of the most strongly associated signals in an independent cohort of 528 GPA cases and 1,228 controls.

Results: Genome-wide significant associations were identified in 32 single-nucleotide polymorphic (SNP) markers across the HLA region, the majority of which were located in the HLA-DPB1 and HLA-DPA1 genes encoding the class II major histocompatibility complex (MHC) DPβ chain 1 and DPα chain 1 proteins, respectively.

IgA and IgG antineutrophil cytoplasmic antibody engagement of Fc receptor genetic variants influences granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (Wegener's) is a rare autoimmune neutrophil-mediated vasculitis that can cause renal disease and mucosal manifestations. Antineutrophil cytoplasmic antibodies (ANCA) are present in many patients, vary in level over time, and induce neutrophil activation through engagement with Fc receptors (FcRs). Given roles for FcRs in ANCA-mediated neutrophil activation and IgA antibodies in mucosal immunity, we hypothesized that FcR genetics and previously unappreciated IgA ANCA affect clinical presentation.

Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis--a meta-analysis.

Objective: The value of repeated ANCA measurements among patients with an established diagnosis of ANCA-associated vasculitis (AAV) remains controversial. The aim of this study was to explore whether either of the two distinct patterns of ANCA values during remission, a rise in ANCA or persistently positive ANCA, predicted future relapse.

Methods: MEDLINE and EMBASE searches were performed.

Alpha₁-antitrypsin deficiency-related alleles Z and S and the risk of Wegener's granulomatosis.

Objective: Deficiency of α(1) -antitrypsin (α(1) AT) may be a determinant of susceptibility to Wegener's granulomatosis (WG). Several previous, mainly small, case-control studies have shown that 5-27% of patients with WG carried the α(1) AT deficiency Z allele. It is not clear whether the S allele, the other major α(1) AT deficiency variant, is associated with WG.

Progress towards a core set of outcome measures in small-vessel vasculitis. Report from OMERACT 9.

The past decade has seen a substantial increase in the number and quality of clinical trials of new therapies for vasculitis, including randomized, controlled, multicenter trials that have successfully incorporated measures of disease activity and toxicity. However, because current treatment regimens for severe disease effectively induce initial remission and reduce mortality, future trials will focus on any of several goals including: (a) treatment of mild-moderate disease; (b) prevention of chronic damage; (c) reduction in treatment toxicity; or (d) more subtle differences in remission induction or maintenance. Thus, new trials will require outcome measure instruments that are more precise and are better able to detect effective treatments for different disease states and measure chronic manifestations of disease.

Epidemiological features of Wegener's granulomatosis and microscopic polyangiitis: two diseases or one 'anti-neutrophil cytoplasm antibodies-associated vasculitis' entity?

Because of their multiple overlapping clinical characteristics, Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) have increasingly been conceptualized as different expressions of a unique anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis (AAV) disease spectrum. However, this continuum theory remains hindered by uncertainty surrounding a potentially common etiology. This review sheds light on our current understanding of the epidemiology of WG and MPA with the aim of weighing the evidence supporting whether or not these two vasculitis forms are distinct diseases.

Assessment of the item selection and weighting in the Birmingham vasculitis activity score for Wegener's granulomatosis.

Objective: To assess the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) with respect to its selection and weighting of items.

Methods: This study used the BVAS/WG data from the Wegener's Granulomatosis Etanercept Trial. The scoring frequencies of the 34 predefined items and any "other" items added by clinicians were calculated.

Hypotheses on the etiology of antineutrophil cytoplasmic autoantibody associated vasculitis: the cause is hidden, but the result is known.

The first description of what is now known as antineutrophil cytoplasmic autoantibody-associated necrotizing vasculitis appeared more than 140 yr ago. Since then, many aspects of the pathogenic pathway have been elucidated, indicating the involvement of antineutrophil cytoplasmic autoantibodies, but why antineutrophil cytoplasmic autoantibodies are produced in the first place remains unknown. Over the years, many hypotheses have emerged addressing the etiology of antineutrophil cytoplasmic antibody production, but no exclusive factor or set of factors can so far be held responsible.

Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis.

Objective: To reevaluate the efficacy and safety of adjunctive low-dose methotrexate (MTX) in giant cell arteritis (GCA).

Methods: An individual patient data meta-analysis of 3 randomized placebo-controlled trials in patients with newly diagnosed GCA was performed. Treatment consisted of initial high-dose corticosteroids and randomly assigned oral MTX therapy (7.

Antiendothelial cell antibodies in patients with Wegener's granulomatosis: prevalence and correlation with disease activity and manifestations.

Objective: Previous studies in small cohorts of patients with Wegener's granulomatosis (WG) or antineutrophil cytoplasmic antibody (ANCA) associated vasculitis have yielded conflicting data regarding the prevalence of antiendothelial cell antibodies (AECA), ranging from 8% to 100%, and the use of AECA as a measure of disease activity. We examined a large, well-characterized cohort of patients with WG and active disease for the presence of AECA.

Methods: Serum from subjects with WG who participated in a clinical therapeutic trial was collected at baseline, when all subjects had active disease.