Publications by authors named "Thibaud Chazal"
Efficacy and safety of targeted therapies in VEXAS syndrome: retrospective study from the FRENVEX.
Ann Rheum Dis
September 2024
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.
Article Synopsis
- - The study investigates the link between eosinophilia and ocular vascular issues, highlighting the procoagulant effects of eosinophils, which can lead to various cardiovascular problems and eye conditions.
- - A retrospective, multicenter analysis of 57 patients revealed that the majority experienced eye issues as the first sign of eosinophil-related diseases, with specific conditions like central retinal artery occlusion being common among them.
- - The findings indicate a poor visual prognosis for these patients, with only 10% achieving full recovery, suggesting the need for urgent treatment and further research on eosinophilia's impacts.
Objective: To evaluate the reproducibility of vessel wall magnetic resonance imaging (VW-MRI) in diagnosing giant cell arteritis (GCA) among groups of radiologists with varying levels of expertise.
Methods: This institutional review board-approved retrospective single-center study recruited patients with suspected GCA between December 2014 and September 2021. Patients underwent 3 -T VW-MRI before temporal artery biopsy.
Characteristics and Prognosis of Binocular Diplopia in Patients With Giant Cell Arteritis.
J Neuroophthalmol
March 2024
Background: Giant cell arteritis (GCA) is a large vessel vasculitis associated with a risk of permanent ophthalmologic complications. Data about diplopia prognosis in GCA are scarce. This study was designed to better characterize diplopia in newly diagnosed GCA patients.
View Article and Find Full Text PDFANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.
Rheumatology (Oxford)
February 2024
Objectives: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests.
Methods: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests.
Erdheim-Chester disease (ECD) is a rare form of L group histiocytosis, accounting for up to 1500 cases to date worldwide, which mainly affects men between their 5th and 7th decade of life. The most frequent manifestations are bone involvement, perirenal infiltration with an evocating appearance of "hairy kidneys", and a "coated aorta" aspect. Lung involvement in ECD is less common and includes pleural infiltration and interstitial lung disease.
View Article and Find Full Text PDFErdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that frequently infiltrates the peri-kidney space ("hairy kidney" appearance), kidney pelvis and proximal ureters, leading to obstructive uropathy. Here, we analyzed the clinical characteristics, imaging findings and long-term kidney outcome of a large multicenter cohort comprising 195 consecutive patients with ECD. Retroperitoneal peri-kidney or peri-ureteral involvement was detected at diagnosis in 147 patients.
View Article and Find Full Text PDFScalp necrosis during a giant cell arteritis relapse triggered by a VZV infection.
Rheumatology (Oxford)
May 2023
Immune checkpoint inhibitors (CPIs) have recently become a cornerstone for the treatment of different advanced cancers. These drugs have the ability to reactivate the immune system against tumour cells but can also trigger a myriad of side effects, termed immune-related adverse events (irAEs). Although there are numerous reports of CPI-related endocrinopathies, hypercalcaemia as a suspected irAE is not well documented.
View Article and Find Full Text PDFArticle Synopsis
- Cardiac sarcoidosis (CS) is often undiagnosed and can lead to severe heart failure, necessitating transplantation without prior identification of the condition.
- A study involving 15 patients with explanted hearts revealed that most were misdiagnosed with other heart issues yet showed signs of sarcoidosis through imaging and pathology.
- Post-transplant outcomes were generally positive, with 14 patients alive after a median follow-up of 28.8 months; however, one patient faced complications, highlighting the need for better pre-transplant diagnoses.
Giant-cell arteritis-related mortality in France: A multiple-cause-of-death analysis.
Autoimmun Rev
December 2018
Objectives: Giant-cell arteritis (GCA) is a large vessel vasculitis. Data regarding mortality are controversial. We describe the mortality data of the French death certificates for the period of 2005 to 2014.
View Article and Find Full Text PDFObjectives: Takayasu arteritis (TA) is a giant cell arteritis usually affecting young women and characterized by inflammatory and ischemic signs of large vessel involvement, including extracranial cerebral arteries. The impact of stroke on TA prognosis has not been well evaluated.
Methods: We performed a retrospective multicenter review of patients with definite TA who experienced at least one stroke and compared the findings to 17 matched patients with TA diagnosis without neurological involvement.