S. Humbert
A5081202033- Venous Thromboembolism Diagnosis and Management
- Vasculitis and related conditions
- Histiocytic Disorders and Treatments
- Eosinophilic Disorders and Syndromes
- Immunodeficiency and Autoimmune Disorders
- Otitis Media and Relapsing Polychondritis
- Parvovirus B19 Infection Studies
- Renal Diseases and Glomerulopathies
- Kawasaki Disease and Coronary Complications
- Atrial Fibrillation Management and Outcomes
- Platelet Disorders and Treatments
- Autoimmune Bullous Skin Diseases
- Blood groups and transfusion
- Autoimmune and Inflammatory Disorders Research
- Vascular Anomalies and Treatments
- IgG4-Related and Inflammatory Diseases
- Inflammatory Myopathies and Dermatomyositis
- Acute Myocardial Infarction Research
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Systemic Sclerosis and Related Diseases
- Blood disorders and treatments
- Cardiovascular Issues in Pregnancy
- Coronary Artery Anomalies
- Skin Diseases and Diabetes
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
Centre Hospitalier Universitaire de Besançon
2016-2025
Assistance Publique – Hôpitaux de Paris
2019
École Polytechnique Fédérale de Lausanne
2019
Centre de Référence Déficits Immunitaires Héréditaires
2019
Center for Rheumatology
2019
Institut des Maladies Génétiques Imagine
2019
Inserm
2015-2019
Collège de France
2019
IFP Énergies nouvelles
2018
Hôpital Saint Eloi
2011-2016
A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS ('Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome').To describe clinical characteristics, laboratory findings outcomes syndrome.One hundred sixteen patients with were referred a French multicentre registry between November 2020 May 2021. The frequency median parameters vital status, from diagnosis the end follow-up, recorded.The main features found be skin lesions...
Patients with biallelic loss-of-function variants of AIRE suffer from autoimmune polyendocrine syndrome type-1 (APS-1) and produce a broad range autoantibodies (auto-Abs), including circulating auto-Abs neutralizing most type I interferons (IFNs). These were recently reported to account for at least 10% cases life-threatening COVID-19 pneumonia in the general population. We report 22 APS-1 patients 21 kindreds seven countries, aged between 8 48 yr infected SARS-CoV-2 since February 2020. The...
<h3>Objectives</h3> The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well the low frequency muscle signs in anti–melanoma differentiation-associated gene 5 antibody–positive (anti-MDA5+) dermatomyositis caused us to question term myositis-specific antibody for anti-MDA5 antibody, homogeneity disease. <h3>Methods</h3> To characterize anti-MDA5+ phenotype, an unsupervised analysis was performed on patients (n = 83/121) and...
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an acquired autoinflammatory monogenic disease with a poor prognosis whose determinants are not well understood. We aimed to describe serious infectious complications and their potential risk factors. Retrospective multicentre study including patients VEXAS from the French Registry. Episodes of infections were described, factors analysed using multivariable Cox proportional hazards models. Seventy-four 133 included....
To assess the safety and efficacy of TNF-α antagonists tocilizumab in patients with Takayasu arteritis (TAK). A total 209 TAK [median age 29 years (interquartile range 7-62)], 186 (89%) females] were included. They received either [n = 132 (63%) 172 lines; infliximab (n 109), adalimumab 45), golimumab 8), certolizumab 6) etanercept 5)] or 77 (37%) 121 i.v. s.c. 95 26 cases, respectively]. complete response at 6 months was evidenced 101/152 (66%) on 75/107 (70%) tocilizumab. Age ≥30 [odds...
Objective. Coexistence of ANCA-associated vasculitis (AAV) and IBD is a rare condition that rarely described in the literature. The aim study was to describe main characteristics patients presenting with both AAV. Methods. A retrospective AAV French Vasculitis Study Group cohort who also had diagnosis conducted. We reviewed medical records outcomes these patients. Results. identified 11 IBD. Four eosinophilic granulomatosis polyangiitis (Churg–Strauss) ulcerative colitis seven (GPA) Crohn's...
The risk of immune thrombocytopenia (ITP) worsening during pregnancy and neonatal ITP (NITP) have never been prospectively studied. We included 180 pregnant 168 nonpregnant women with in a prospective, multicenter, observational cohort study. A total 131 were matched to by history splenectomy, status (no response, complete response), duration. Groups followed for 15 months. primary outcome was the first occurrence defined composite end point including bleeding events and/or severe (<30 ×...
The aim of this study was to determine the characteristics, treatment, and outcome according each etiology pachymeningitis. We conducted a retrospective multicenter French nationwide between 2000 2016 describe outcome, treatment included 60 patients (median age 55.5 years; interquartile range [IQR] 30–80, female/male ratio 0.43). Neurologic signs were present in 59 (98%) consisted headache 43 (72%), cranial nerve palsy 33 (55%), confusion 10 (17%), seizures 7 (12%), focal neurologic 9 (15%)....
GCA is a large vessel vasculitis for which triggering factors remain unknown. Clonal haematopoiesis (CH) was associated with atherosclerosis through the induction of inflammation in myeloid cells, and data suggest that CH expansion may support each other to induce pro-inflammatory loop. Our objective describe impact JAK2p.V617F-mutated myeloproliferative neoplasms (MPNs) on screen MPN-free patients mutations.We performed retrospective case-control study comparing characteristics 21 MPN 42...
In this large multicentre study, we compared the effectiveness and safety of tocilizumab intravenous versus subcutaneous (SC) in 109 Takayasu arteritis (TAK) patients.We conducted a retrospective study referral centres from France, Italy, Spain, Armenia, Israel, Japan, Tunisia Russia regarding biological-targeted therapies TAK, since January 2017 to September 2019.A total TAK patients received at least 3 months therapy were included study. Among them, 91 18 SC tocilizumab, respectively. A...
<h3>Background</h3> The PEXIVAS (Plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis) trial showed that a reduced-dose glucocorticoid regimen (redGC) was non-inferior to standard-dose (standGC) with respect death or end-stage kidney disease (ESKD) patients ANCA-associated vasculitis (AAV). However, the primary endpoint did not include progression relapse, cyclophosphamide main induction therapy rituximab (RTX)-treated tended have...