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.
Objective: Elevated inflammatory markers play a crucial role in the diagnosis and follow-up of patients with giant cell arteritis (GCA). This study aimed to describe the prevalence, characteristics, and outcomes of patients with low baseline (< 10 mg/L) C-reactive protein (CRP) in GCA.
Methods: A retrospective observational study was conducted at Lille University Hospital, involving all patients diagnosed with GCA between January 2000 and April 2023.
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an eosinophilic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Rituximab has emerged as the standard of care in other types of ANCA-associated vasculitis, but controlled studies on its use in EGPA are yet lacking.
Objective: To compare rituximab with conventional strategy for the induction of remission in patients with EGPA.
Innate immune cells appear to have an important implication in the resolution and/or the aggravation of the COVID-19 pathogenesis after infection with SARS-CoV-2. To better appreciate the role of these cells during COVID-19, changes in blood eosinophil, the neutrophil and monocyte count, and levels of surface protein markers have been reported. However, analyses at several timepoints of multiple surface markers on granulocytes and monocytes over a period of one month after a SARS-CoV-2 infection are missing.
View Article and Find Full Text PDFBackground: Patients with antiphospholipid syndrome (APS) display a wide range of clinical manifestations with similar immunological profile with the presence of lupus anticoagulant in around 70% of them. Although antiphospholipid antibodies (aPL) profile influences the risk of thrombosis in APS, APS risk stratification remains to be improved.
Objectives: Our study aimed to evaluate thrombin generation test (TGT) interest to improve disease severity stratification in APS patients.
Background: Monoclonal gammopathy-associated capillary leak syndrome (MG-CLS) is a rare condition characterized by recurrent episodes of hypovolemic shock caused by a sudden increase in capillary permeability. The COVID-19 pandemic has been associated with a rise in MG-CLS episodes and increased mortality. We aimed to explore the association between MG-CLS and SARS-CoV-2 infection.
View Article and Find Full Text PDFRheumatology (Oxford)
August 2025
Objective: Antiphospholipid syndrome (APS) is an acquired autoimmune prothrombotic condition. Vascular liver disorders (VLD), such as portal vein thrombosis (PVT), Budd-Chiari syndrome (BCS) and porto-sinusoidal vascular disorder (PSVD), are rare and related to an underlying hypercoagulable state in most cases. We aimed to describe the clinical and immunological features of APS patients with VLD.
View Article and Find Full Text PDFBackground: Vascular Ehlers-Danlos syndrome is a rare genetic disorder characterized by defective type III collagen and a high risk of arterial morbidity and mortality. Several cardiovascular drugs are used for treatment, including celiprolol, but no controlled trial in this condition has been conducted to date. We hypothesized the benefit of the addition of an angiotensin II receptor blocker.
View Article and Find Full Text PDFRev Med Interne
January 2025
Skin Health Dis
August 2024
Objectives: We aimed to compare clinical spectrum and outcome between adults and children with Takayasu's arteritis (TAK) in a European population.
Methods: We made a nationwide retrospective observational study between 1988 and 2019. All adult patients met the ACR diagnostic criteria for TAK and all children met the EULAR/PRINTO/PRES criteria for paediatric TAK.
Introduction: Rare disease referral centres are entrusted with missions of clinical expertise and research, two activities that have to contend with numerous obstacles. Providing specialist opinions is time-consuming, uncompensated and limited by difficulties in exchanging medical data. Clinical research is constrained by the need for frequent research protocol visits.
View Article and Find Full Text PDFBackground: Cerebrovascular ischemic events (CIE) are among the most severe complications of giant cell arteritis (GCA). Heterogeneity between different studies in the definition of GCA-related CIE leads to uncertainty regarding their real prevalence. The aim of our study was to evaluate the prevalence and describe the characteristics of GCA-related CIE in a well-phenotyped cohort completed by a meta-analysis of the existing literature.
View Article and Find Full Text PDFObjectives: To evaluate whether inflammatory and complement biomarkers are associated with specific characteristics of antiphospholipid syndrome (APS).
Methods: Serum levels of interleukin (IL)-1β (IL-1β), IL-6, IL-8, IL-10, tumour necrosis factor (TNF)-α, interferon-α (IFN)-α, IFN-γ, vascular endothelial growth factor (VEGF), intercellular adhesion molecule 1 (ICAM-1), E-selectin, and vascular cell adhesion molecule (VCAM)-1, and plasma levels of soluble C5b-9 (sC5b-9), C3a, C4a, Bb fragment were measured in unselected APS patients. Twenty-five healthy blood donors were included as controls.
Background: The prevention of catastrophic antiphospholipid syndrome (CAPS), a rare complication of antiphospholipid syndrome (APS), is a major goal.
Objectives: We analyzed its precipitating factors, focusing on anticoagulation immediately before CAPS episodes.
Methods: We retrospectively analyzed patients in the French multicenter APS/systemic lupus erythematosus database with at least 1 CAPS episode.
Importance: Catastrophic antiphospholipid syndrome (CAPS) is a severe, rare complication of antiphospholipid syndrome (APS), but cutaneous involvement has not yet been adequately described.
Objective: To describe cutaneous involvement during CAPS, its clinical and pathological features, and outcomes.
Design, Setting, And Participants: This cohort study was a retrospective analysis of patients included in the French multicenter APS/systemic lupus erythematosus register (ClinicalTrials.
(1) Background: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease with a high mortality and morbidity rate. Identification of biomarkers that can predict the evolution of SSc is a key factor in the management of patients. The aim of this study was to assess the association of routine laboratory parameters, widely used in practice and easily available, with the severity and progression of SSc.
View Article and Find Full Text PDFBackground: Antiphospholipid syndrome (APS) nephropathy (APSN) is a rare pattern with specific features resulting from microvascular lesions. The prognosis of APSN, outside of lupus nephritis, is unknown. The aim of this study was to describe the renal, vascular and overall outcomes of patients with APSN.
View Article and Find Full Text PDFJ Allergy Clin Immunol Pract
November 2022
Background: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown.
Objective: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS.