Achille Aouba
A5006955984- Vasculitis and related conditions
- Otitis Media and Relapsing Polychondritis
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Sarcoidosis and Beryllium Toxicity Research
- Systemic Lupus Erythematosus Research
- Inflammasome and immune disorders
- Urticaria and Related Conditions
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Eosinophilic Disorders and Syndromes
- Renal Diseases and Glomerulopathies
- Histiocytic Disorders and Treatments
- Autoimmune and Inflammatory Disorders Research
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Immunodeficiency and Autoimmune Disorders
- Platelet Disorders and Treatments
- Atherosclerosis and Cardiovascular Diseases
- Vascular Anomalies and Treatments
- interferon and immune responses
- Autoimmune Bullous Skin Diseases
- Hemophilia Treatment and Research
- Cell Adhesion Molecules Research
- Kawasaki Disease and Coronary Complications
- Mast cells and histamine
- Multiple Myeloma Research and Treatments
- NF-κB Signaling Pathways
Université de Caen Normandie
2016-2025
Centre Hospitalier Universitaire de Caen Normandie
2016-2025
Normandie Université
2016-2025
Centre Hospitalier Universitaire de Nice
2018-2025
Université Paris Cité
2008-2018
Inserm
2011-2018
Université de Montpellier
2018
Saint Joseph Hospital
2018
Mount Sinai Hospital
2018
Hôpital Cochin
2005-2017
Abstract Objective Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg‐Strauss) (EGPA), limited patient numbers and followup durations, demonstrated that clinical presentation at diagnosis, but not outcome, differed according to antineutrophil cytoplasmic antibody (ANCA) status. This study was undertaken describe the main characteristics a larger cohort their long‐term outcomes. Methods A retrospective EGPA patients in French Vasculitis Study Group who satisfied American...
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...
Azacitidine can be effective in myelodysplastic syndromes (MDS) associated with inflammatory/autoimmune diseases. Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) is a new monogenic autoinflammatory caused by somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutation, often MDS, whose treatment difficult and not yet codified. Based on French nationwide registry of 116 patients VEXAS, we report the efficacy safety azacitidine 11 VEXAS MDS. Clinical response...
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.
To investigate the safety and efficacy of rituximab (RTX) for systemic symptoms in patients with primary Sjögren's syndrome (pSS), changes B cell biomarkers.The records 16 pSS according to American European consensus group criteria were reviewed retrospectively.Patients, all women, had a median age 58.5 (range 41-71) years disease duration 9.5 0-25) years. RTX was prescribed lymphoma (n = 5), refractory pulmonary polysynovitis 2), severe mixed cryoglobulinaemia thrombocytopenia 1)...
Previous studies reported a 2- to 17-fold higher risk of aortic complications (dilation or dissection) in patients with giant-cell arteritis (GCA). We aimed determine whether not GCA large-vessel involvement demonstrated by positron emission tomography 18F-fluorodeoxyglucose combined computed (FDG-PET/CT) have complications. conducted retrospective multicenter study between 1995 and 2014. Patients were included if they fulfilled at least 3 American College Rheumatology criteria for GCA, 2...
Objective. Our aim was to describe patients with giant cell arteritis (GCA)–related stroke and compare them a control group of GCA without stroke. Methods. We created retrospective multicenter cohort (1) diagnosed according the American College Rheumatology criteria between 1995 2015, (2) occurring at time diagnosis or within 4 weeks starting therapy. The consisted Results. Forty [21 women (53%), median age 78 (60–91) yrs] GCA-related were included compared 200 patients. Stroke occurred in...
Objective To describe the efficacy and safety of biologics for treatment eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective European collaborative study was conducted in patients EGPA who received refractory and/or relapsing disease. Results Among 147 included study, 63 rituximab (RTX), 51 mepolizumab (MEPO), 33 omalizumab (OMA). At time inclusion, median Birmingham Vasculitis Activity Score (BVAS) 8.5 (interquartile range [IQR] 5–13) RTX group, while BVAS OMA...
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....
Background. The crowned dens syndrome, related to microcrystalline deposition in the peri-odontoid articular and abarticular structures, is mainly responsible for acute or chronic cervical pain.
<h3>Objectives</h3> To further improve the distinction between primary angiitis of CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS). <h3>Methods</h3> We compared 2 large French cohorts patients with PACNS (n = 110, retrospectively prospectively enrolled) RCVS 173, enrolled). <h3>Results</h3> Patients were predominantly female (<i>p</i> < 0.0001), migraines more often exposed to vasoactive substances 0.0001) or postpartum 0.002) than PACNS. Headache, especially...
Vinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this could be extended to adults LCH questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% multisystem LCH) who were treated vinblastine + steroids as a first-line chemotherapy and followed median time of 83 months. The objectives determine overall response rate (based on Histiocyte Society criteria), disease reactivation rate,...
Objective. To identify characteristics and factors associated with relapse glucocorticoid (GC) dependence in patients giant cell arteritis (GCA). Methods. We retrospectively analyzed 326 consecutive GCA followed for at least 12 months. Factors GC were identified multivariable analyses. Results. The (73% women) up 62 (12–262) During followup, 171 (52%) relapsed, including 113 (35%) who developed dependence. Relapsing had less history of stroke (p = 0.01) presented large-vessel vasculitis...
In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis polyangiitis (EGPA) failed lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim this study was analyse longer-term outcomes determine whether subsequent (IARE) rates differed.After M24, were followed prospectively, being treated based on physicians'...