Effectiveness of intravenous infliximab versus subcutaneous adalimumab in Takayasu arteritis: multicenter retrospective study.

Objectives: In this large multicentre study, we aimed to compare the effectiveness of intravenous infliximab vs subcutaneous adalimumab in patients with Takayasu arteritis.

Methods: We conducted a retrospective multicentre study across referral centers in France, Italy, Spain, Armenia, Israel, Japan, Tunisia, and Russia, analyzing biological-targeted therapies in TAK from January 2017 to September 2019.

Results: A total of 135 TAK patients who received adalimumab (n = 34) or infliximab (n = 101) for at least 3 months were included.

Comparing the efficacy and safety of cyclophosphamide, infliximab and methotrexate in neurosarcoidosis: a multicenter retrospective study.

Introduction: Neurosarcoidosis is a granulomatous disease affecting 10% of patients with sarcoidosis. In lack of standardized guidelines, various therapeutic strategies exist. Beyond corticosteroids, cyclophosphamide, infliximab, or methotrexate are available options.

[Assessment of internship sites in Internal Medicine and Clinical Immunology, 7 years after the Residency Training Program Reform: Findings from a 2024 year-end survey in France].

Objective: To compile a list of approved training sites for the Residency Training Program [Diplôme d'Études Spécialisées] in Internal Medicine and Clinical Immunology (DES-MIIC) in France.

Method: All local coordinators of the DES-MIIC were contacted to establish the list of approved internship sites for the MIIC DES within their geographical subdivision.

Results: We listed 244 approved training sites, of which 87 (35.

Four months of treatment with anakinra combined with glucocorticoids for giant cell arteritis: a multicenter, randomized, double-blind, placebo-controlled trial.

Background: Efficacy and tolerance of anakinra (ANK) in the treatment of giant cell arteritis (GCA) need to be assessed.

Methods: This phase 3 study (NCT02902731) was a prospective multicenter, randomized, double-blind, placebo-controlled trial conducted over a 52-week period. GCA patients were randomized 1:1.

Efficacy and glucocorticoid sparing of dupilumab in IgG4-related disease: a case series and literature review.

Objectives: IgG4-related disease (IgG4-RD) is a multisystem fibro-inflammatory disorder for which glucocorticoids (GC) represent the initial therapeutic intervention. Second-line treatments are not currently codified. The use of dupilumab, an inhibitor of interleukin (IL)-4 and IL-13 signalling, has recently been described as a potential alternative treatment.

Cluster analysis reveals the clinical spectrum of Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a clonal-inflammatory neoplasm driven by mutations in MAPK pathway proto-oncogenes, such as BRAF. Clinical manifestations are protean, affecting virtually every system. This cohort study analyzed 661 patients with ECD to classify them based on clinical features and mutational profiles using unsupervised clustering.

Efficacy and safety of azacitidine for VEXAS syndrome: a large-scale retrospective study from the FRENVEX group.

VEXAS (Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic) syndrome is a severe monogenic disorder caused by somatic UBA1 mutations, characterized by inflammation, cytopenias and frequent association with myelodysplastic neoplasms (MDS). Steroid dependence is common, and targeted therapies have demonstrated limited efficacy. Azacitidine (AZA), a hypomethylating agent used in MDS, has shown potential in VEXAS but data remain limited.

Data-Driven Clustering Approach to Identify Different Phenotypes of Primary Central Nervous System Vasculitis.

Background: To determine whether hierarchical unsupervised cluster analysis identifies a phenotypic distinction in adult patients with primary CNS vasculitis (PCNSV).

Methods: An agglomerative hierarchical cluster analysis based on the Ward method was conducted, including 153 patients with complete baseline phenotypic characterization in the COVAC' registry.

Results: The hierarchical analysis identified two main clusters.

Characteristics and outcomes of adult patients with familial Mediterranean fever: Comparison of patients with one versus two pathogenic exon 10 MEFV mutations.

Objective: To describe the main features and outcomes of a large cohort of adult familial Mediterranean fever (FMF) patients with one pathogenic MEFV mutations and compare them to FMF patients displaying 2 pathogenic MEFV mutations.

Methods: In a retrospective single-referential French center cohort of 581 patients with FMF, 178 FMF patients with one pathogenic mutation were retrieved and compared to 403 patients with 2 pathogenic MEFV mutations. The diagnosis of FMF was based on the Eurofever/PRINTO classification criteria for all patients, and they had all been sequenced for MEFV.

A20 haploinsufficiency diagnosis beyond systemic lupus erythematosus: A systematic review of the literature.

Systemic lupus erythematosus (SLE) is an autoimmune disease whose pathophysiology remains incompletely understood, involving genetic and epigenetic factors. However, an increasing small subset of patients present with monogenic lupus, providing insight into the pathogenesis of the disease. This systematic review focuses on SLE associated with A20 haploinsufficiency (HA20), a monogenic disorder associated with tumor necrosis factor alpha-induced protein 3 gene (TNFAIP3) variants.

Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis.

Background And Aims: Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments.

Efficacy and safety of targeted therapies in VEXAS syndrome: retrospective study from the FRENVEX.

Objectives: Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic (VEXAS) syndrome is an adult-onset autoinflammatory disease associated with somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutations. We aimed to evaluate the efficacy and safety of targeted therapies.

Methods: Multicentre retrospective study including patients with genetically proven VEXAS syndrome who had received at least one targeted therapy.

Autoantibodies against complement proteins in patients with antiphospholipid syndrome: Prevalence and clinical associations.

Seventy-seven patients with antiphospholipid syndrome were tested for autoantibodies against C1q, C3, FB, FH, and C4bp. Fifty-seven patients had at least one anti-complement antibody. IgM anti-FH positivity was associated with thrombosis when anti-C3 and anti-FB were, negatively or positively, associated with various noncriteria manifestations of antiphospholipid syndrome.

VEXAS syndrome: A new mimicker of idiopathic multicentric Castleman disease.

Introduction: Idiopathic Multicentric Castleman Disease (iMCD) is a complex and poorly understood pathophysiological entity, which encompasses a variety of conditions and can mimic or be associated with autoimmune/autoinflammatory diseases, making it challenging to diagnose and treat. Vacuoles, Enzyme E1, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome is an adult-onset autoinflammatory disorder associated with hematological abnormalities and caused by acquired somatic mutations in the ubiquitin-like modifier activating enzyme 1 gene (UBA1) which shares several common clinical and biological signs with iMCD. In this article, we report a patient with VEXAS syndrome initially presenting as iMCD, questioning the link between these two entities.

Histological characterization of liver involvement in systemic mastocytosis.

Background And Aims: Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences of aggressive SM diagnosis. We aimed to describe the histological features associated with liver SM using updated tools.

Frequency and characteristics of severe relapses in giant cell arteritis.

Objectives: To assess the frequency and characteristics of severe relapse in patients with GCA in a real-life setting.

Methods: In a monocentric database of 530 patients, we retrospectively analysed patients who experienced at least one relapse and distinguished severe from non-severe relapses. Severe relapse was defined by the occurrence of an ischaemic event (ophthalmologic, neurologic, digestive, limb ischaemia), the occurrence of an aortic complication (i.

Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients.

Introduction: This study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.

Methods: Among a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.