Publications by authors named "Benjamin De Sainte-Marie"
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.
Objective: Describe the characteristics of patients presenting with TTS during the course of a broad spectrum of systemic diseases, in comparison to classic TTS.
Methods: French multicenter retrospective case-control study completed by a literature review.
Results: 19 new cases were included in the study.
Introduction: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect almost any organ. IgG4-related ophthalmic disease is a protean condition involving the orbit and ocular adnexa. Although a few cases of uveitis have been reported, the exact pattern of IgG4-related intraocular manifestations remains unclear.
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- * Conducted in three French hospitals from February 2021 to February 2022, the research analyzed the outcomes of 235 hospitalized COVID-19 patients, matching them to reduce bias in treatment allocation.
- * Results indicated that both medications had similar effects on 28-day mortality rates, in-hospital mortality, oxygen requirements, ICU admissions, and secondary infection rates, suggesting comparable safety and efficacy.
Background: The vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a newly identified autoinflammatory disorder related to somatic UBA1 mutations. Up to 72% of patients may show lung involvement.
Research Question: What are the pleuropulmonary manifestations in VEXAS syndrome?
Study Design And Methods: One hundred fourteen patients were included in the French cohort of VEXAS syndrome between November 2020 and May 2021.
Objective: Patients with inflammatory myopathies (IM) are known to have an increased risk of developing malignancies. Autoimmune and inflammatory diseases occur in up to 25% of patients with myelodysplastic syndrome (MDS). This study aimed to describe the rare association between IM and MDS.
View Article and Find Full Text PDFIgG4-related disease is a chronic inflammatory disease characterized by clinical, biological and pathological unifying findings. Because these criteria are not always all together available in patients and because biological and pathological markers are not totally specific, the diagnosis should be retained after exclusion of mimickers. Since the individualization of IgG4-RD, several studies have allowed to better characterize immunological abnormalities associated with this particular condition.
View Article and Find Full Text PDFBackground: Fat infiltration in individual muscles of sporadic inclusion body myositis (sIBM) patients has rarely been assessed.
Methods: Sixteen sIBM patients were assessed using MRI of the thighs and lower legs (LL). The severity of fat infiltration, proximal-to-distal and side asymmetries, and the correlations with clinical and functional parameters were investigated.