R. Bourguiba
A5045829349- Inflammasome and immune disorders
- Otitis Media and Relapsing Polychondritis
- Salivary Gland Disorders and Functions
- COVID-19 Clinical Research Studies
- Systemic Sclerosis and Related Diseases
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Peptidase Inhibition and Analysis
- Eosinophilic Esophagitis
- IL-33, ST2, and ILC Pathways
- Vascular Anomalies and Treatments
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Salivary Gland Tumors Diagnosis and Treatment
- interferon and immune responses
- Gout, Hyperuricemia, Uric Acid
- Peripheral Neuropathies and Disorders
- Autoimmune and Inflammatory Disorders Research
- Long-Term Effects of COVID-19
- Kawasaki Disease and Coronary Complications
- IgG4-Related and Inflammatory Diseases
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Vasculitis and related conditions
- Ocular Diseases and Behçet’s Syndrome
- SARS-CoV-2 and COVID-19 Research
- Retinal and Optic Conditions
- Liver Disease Diagnosis and Treatment
Hôpital Tenon
2019-2025
Sorbonne Université
2019-2025
Assistance Publique – Hôpitaux de Paris
2019-2025
Centre de Référence des Maladies Autoinflammatoires et des Amyloses
2020-2025
Hôpital Mongi Slim
2016-2025
Tunis El Manar University
2024-2025
Security Forces Hospital
2025
Faculté de médecine de Tunis
2024
Tunis University
2024
Hôpital André Mignot
2022-2024
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...
Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic (VEXAS) syndrome is an adult-onset disease associated with ubiquitin-like modifier-activating enzyme 1 (UBA1) mutations. We aimed to evaluate the efficacy safety of targeted therapies.
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....
ABSTRACT Background Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, associated with MEFV mutations. FMF patients can experience liver involvement, potentially leading to cirrhosis. Objectives This study aimed evaluate involvement in at a French tertiary centre for adult FMF. Methods We conducted an observational displaying 2 pathogenic mutations National Reference Center Autoinflammatory Diseases and Inflammatory Amyloidosis (CEREMAIA) Paris included...
Abstract Background/Aims Fibromyalgia is a complex musculoskeletal disorder characterized by widespread chronic pain along with various psychosomatic manifestations that impact the quality of life patients. This condition often observed in several inflammatory diseases, particularly bowel diseases (IBD). Our study aims to determine prevalence fibromyalgia patients IBD as well predictive factors order provide better clinical management for affected these two conditions. Methods...
Abstract Background/Aims Chronic inflammatory bowel disease (IBD) can have extra-intestinal manifestations, notably osteoarticular manifestations such as sacroiliitis (SI). It may be symptomatic, inaugurating associated spondyloarthritis, or asymptomatic, consistent with isolated SI. The aim of this study is to investigate the prevalence and clinical radiographic characteristics SI in IBD. Methods This was a retrospective patients treated for Crohn’s (CD) ulcerative colitis (UC)....
There is little known about SARS-CoV-2 infection in patients with systemic autoinflammatory disease (SAID). This study aimed to describe epidemiological features associated severe form and death. Mortality between without SAID hospitalised for was compared.A national multicentric prospective cohort conducted from the French Rheumatic Musculoskeletal Diseases (RMD) COVID-19 cohort. Patients were matched non-SAID on age±7 years, gender number of comorbidities consider important confounding...
This case series reports on the clinical presentation, laboratory findings, imaging characteristics, treatments, and outcomes of nonischemic cardiac manifestations in patients with VEXAS syndrome confirmed pathogenic UBA1 alterations.
Familial Mediterranean fever (FMF) patients may have hepatic cytolysis, although its origin is not formally elucidated. We aimed to evaluate liver involvement in familial using non-invasive methods. All adult FMF harboring two non-ambiguous mutations of the MEFV gene with cytolysis were identified a French tertiary center for FMF. Liver impairment was explored FibroMax (a method estimate steatosis, necrosis, inflammation and fibrosis) ultrasound. Among 520 patients, 43 had persistent 20...
<h3>Background</h3> Familial Mediterranean fever (FMF) is the most common autoinflammatory disease worldwide. It affects mainly population from origin and associated with <i>MEFV</i> exon 10 mutations. FMF characterized by short recurrent attacks of fever, abdominal or thoracic pain that lasts less than three days [1]. Several studies reported diagnosis may be missed delayed even in countries a high prevalence such Turkey Israel but real causes explaining diagnostic delay are not totally...