Background: Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample-size and/or follow-up duration are available.
Methods: The COHESion study is a national prospective multicenter multidisciplinary cohort recruiting both adults or children with the spectrum of eosinophilic disorders (including reactive HE/HES [HE/HES-R], idiopathic HES [HES-I], lymphocytic HES [HES-L], neoplastic HE/HES [HE/HES-N], HE of unknown significance [HE-US], as well as IgG4-related disease [IgG4RD] or ANCA-negative eosinophilic granulomatosis with polyangiitis [EGPA] overlaps).
Objective: Describe the demographic data and clinical phenotype of cranial palsy induced by immune checkpoint inhibitors (CNP-ICI).
Methods: A systematic literature review of the literature was performed in Pubmed, Web of Science, and Embase, including 68 articles and 136 patients (PROSPERO no. CRD42024517262).
Background: Patients with chronic illnesses, especially rare autoimmune and/or systemic diseases associated with significant diagnostic uncertainty, have a representation of their illness and a sometimes prolonged hospitalization experience that can be traumatic and anxiety-provoking.
Objective: The aim of this study was to evaluate the impact of a non-medicinal medical hypnosis intervention in reducing the stress state and improving the experience of patients hospitalized in an internal medicine department.
Methods: We conducted a prospective study of 24 patients hospitalized in the Internal Medicine Department of Lille University Hospital in 2023.
Background: Scalp, tongue and/or lip necrosis are rare complications of GCA.
Objectives: To describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis.
Methods: A retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period.
Objective: To evaluate rituximab (RTX) resistance at 3months (M3) of induction therapy in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV).
Methods: Multicentre French retrospective study conducted between 2010 and 2020 including patients with newly diagnosed or relapsing AAV (granulomatosis with polyangiitis or microscopic polyangiitis) having received induction therapy with RTX. Primary endpoint was the presence of RTX resistance at 3months (M3) defined as uncontrolled disease (worsening feature on the BVAS/WG 1month after RTX induction) or disease flare (increase in BVAS/WG of≥1 point before M3).
Introduction: Coronaritis is a rare but serious complication of giant-cell arteritis (GCA), with an estimated prevalence of less than 1%, however difficult to establish, and of early onset.
Methods: We describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication.
Results: The first patient presented with stable angina on common trunk coronaritis with ostial stenosis.
(1) Background: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease with a high mortality and morbidity rate. Identification of biomarkers that can predict the evolution of SSc is a key factor in the management of patients. The aim of this study was to assess the association of routine laboratory parameters, widely used in practice and easily available, with the severity and progression of SSc.
View Article and Find Full Text PDFObjectives: Although dyslipidemia is a strong risk factor for thrombosis in antiphospholipid syndrome (APS), it has been poorly studied. This study aimed to assess lipids profile and risk factors for unachieved cholesterol levels in a real-life APS population.
Methods: Inclusion criteria were: APS diagnosis according to international classification criteria, referring to the out-patients clinic of our tertiary care center for their follow-up, and having a blood sample collection for lipids levels determination.
We provide an original multi-stage approach identifying a gene signature to assess murine fibroblast polarization. Prototypic polarizations (inflammatory/fibrotic) were induced by seeded mouse embryonic fibroblasts (MEFs) with TNFα or TGFß1, respectively. The transcriptomic and proteomic profiles were obtained by RNA microarray and LC-MS/MS.
View Article and Find Full Text PDFObjective: There is little known about SARS-CoV-2 infection in patients with systemic autoinflammatory disease (SAID). This study aimed to describe epidemiological features associated with severe disease form and death. Mortality between patients with and without SAID hospitalised for SARS-CoV-2 infection was compared.
View Article and Find Full Text PDFBackground: Interstitial lung disease is a common complication of systemic sclerosis (SSc-ILD), and it remains difficult to accurately predict its course. Progressing ILD could be more metabolically active, suggesting that the F-FDG tracer could be a tool in the managing of SSc-ILD.
Methods: In our center, SSc patients and controls (non-Hodgkin lymphoma cured after first-line regimen) who had received a PET/CT were screened retrospectively.
Introduction: Our objective was to evaluate characteristics, treatment and outcome of vasculitis associated with myelodysplastic syndrome (MDS) and chronic myelomonicytic leukemia (CMML) PATIENTS AND METHODS: Retrospective descriptive analysis of MDS/CMML-related vasculitis and comparison with MDS/CMML patients without dysimmune features.
Results: Seventy patients with vasculitis and MDS/CMML were included, with median age of 71.5 [21-90] years and male/female ratio of 2.
Background: Systemic sclerosis (SSc) is a severe and highly heterogeneous disease. The modified Rodnan skin score (mRSS) is a widely used tool for the assessment of the extent and degree of skin thickness. This study aimed to identify the classes of patients with early similar skin thickening trajectories without any a priori assumptions and study their associations with organ involvement and survival.
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