Publications by authors named "Durel C"

Article Synopsis
  • The study aimed to assess the effectiveness of combining glucocorticoids (GCs) with cyclophosphamide (CYC) for treating patients with poor-prognosis eosinophilic granulomatosis with polyangiitis (EGPA) compared to using GCs alone.
  • Data from a European multicenter database were analyzed, involving 209 patients and focusing on relapse rates and other related outcomes over 12 to 24 months.
  • Results indicated that adding CYC significantly reduced the risk of relapse and related complications compared to GCs alone, suggesting a beneficial role for CYC in the treatment of poor-prognosis EGPA.
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  • Porphyrias are genetic disorders that affect heme production, with acute hepatic porphyrias (AHP) like acute intermittent porphyria (AIP) often mimicking neurological conditions, leading to potential misdiagnoses.
  • A 41-year-old woman experienced symptoms resembling neuralgic amyotrophy but was ultimately diagnosed with AIP after finding elevated levels of porphobilinogen and delta-aminolevulinic acid, following an initial misdiagnosis.
  • This case highlights the importance of considering AIP in patients with acute neuropathies, particularly when they present with abdominal symptoms, to ensure timely and effective treatment.
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Introduction: In 2013, rituximab was approved in France for the treatment of ANCA-associated vasculitis (AAV). The aim of the study was to compare the treatment and health events of adult incident patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), included before rituximab approval (over 2010-2012, Group 1) and those included after rituximab approval (over 2014-2017, Group 2).

Method: Data were extracted from the French National Health Insurance database (SNDS) including outpatient health care consumption and hospital discharge forms.

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Objectives: To evaluate the ability of FDG PET/CT, at diagnosis of giant cell arteritis (GCA) and during follow-up, to predict occurrence of relapse in large-vessel GCA (LV-GCA).

Methods: We conducted a retrospective study using the French Study Group for Large-Vessel Vasculitis (GEFA) network. Data from patients with LV-GCA diagnosed by PET/CT and who had PET/CT in the following year were collected.

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Introduction: Asthma associated with eosinophilic granulomatosis with polyangiitis (EGPA) is often severe and corticosteroid-dependent, leading to significant morbidity. Mepolizumab and benralizumab are humanized monoclonal antibodies targeting interleukin 5 (IL-5) and its receptor, respectively. They have been shown to be effective in steroid-sparing in patients with severe eosinophilic asthma.

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We investigated using a custom NGS panel of 149 genes the mutational landscape of 64 consecutive adult patients with tyrosine kinase fusion-negative hypereosinophilia (HE)/hypereosinophilic syndrome (HES) harboring features suggestive of myeloid neoplasm. At least one mutation was reported in 50/64 (78%) patients (compared to 8/44 (18%) patients with idiopathic HE/HES/HE used as controls; p < .001).

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Article Synopsis
  • This study evaluated the effectiveness and safety of the IL-5 receptor inhibitor benralizumab for treating patients with eosinophilic granulomatosis with polyangiitis (EGPA) across 28 European centers.
  • Out of 121 patients treated, complete responses increased from 12.4% at 3 months to 46.4% at 12 months, while partial responses decreased over the same period.
  • Notable improvements were seen in disease activity, as measured by the Birmingham Vasculitis Activity Score (BVAS), and a reduction in various disease manifestations, alongside better lung function.
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Purpose: To describe the demographic and clinical characteristics of uveitis in patients with giant cell arteritis (GCA), their treatments, and evolution.

Methods: A national retrospective cohort study was performed. The inclusion criteria were as follows: patients with GCA fulfilling the 2022 ACR/EULAR criteria and a diagnostic of uveitis attested by an ophthalmologist.

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Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is often associated with glucocorticoid-dependent asthma and/or ear, nose and throat (ENT) manifestations. When immunosuppressants and/or mepolizumab are ineffective, dupilumab could be an option. We describe the safety and efficacy of off-label use of dupilumab in relapsing and/or refractory EGPA.

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Background: Benralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series.

Methods: We conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab.

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Article Synopsis
  • A retrospective study on polyarteritis nodosa (PAN) analyzed data from 196 patients referred to a French study group between 2005 and 2019, highlighting significant changes in the disease's landscape.
  • Main symptoms included constitutional, neurological, skin, and musculoskeletal issues, with 28% of cases being secondary PAN due to conditions like myelodysplastic syndrome and cancers; most patients (98.5%) were treated with glucocorticoids.
  • The study found high relapse rates associated with older age and specific clinical features, with mortality linked to factors like age, necrotic purpura, kidney injury, and secondary PAN, emphasizing the need for improved patient management.
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Purpose: The study aims to determine the impact of initial management in Vogt-Koyanagi-Harada syndrome (VKHS).

Methods: Patients diagnosed with a VKHS between January 2001 and December 2020 in two French tertiary centers were included in a retrospective study.

Results: Fifty patients were included with a median duration of follow-up of 29.

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Article Synopsis
  • - The study examined the characteristics of granulomatosis with polyangiitis (GPA) in patients who experienced induction failure, focusing on different treatments and how effective salvage therapies were from 2006 to 2021.
  • - A total of 51 patients with GPA and induction failure were analyzed, revealing that those treated with intravenous cyclophosphamide often had more severe symptoms like relapsing disease and orbital masses compared to controls, while those on rituximab showed significant renal complications.
  • - After trying salvage therapies, 69% of patients achieved remission, with a notable success in switching therapies; 50% of those inadequately responding to cyclophosphamide improved with rituximab, while patients progressing
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Objective: Dermatomyositis associated with anti-MDA5 autoantibodies (DM-MDA5+) is a rare autoimmune disease usually characterized by skin involvement, often-severe lung involvement, and general features. Several reports of infections have been described, sometimes early after the introduction of immunosuppressive therapy. We studied the infection risk in a DM-MDA5+ population.

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Background: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are rare systemic necrotizing vasculitis. The national incidence and prevalence of GPA/MPA and patient mortality remain unknown in France. A real-life study using retrospective data from the French National Health Data System was set up to describe the epidemiology and demographic characteristics of hospitalized GPA and MPA patients, overall and by disease.

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Objectives: To describe the effectiveness and safety of biologics for the treatment of relapsing and/or refractory polyarteritis nodosa (PAN).

Methods: A retrospective European collaborative study was conducted in patients with PAN who received biologics for relapsing and/or refractory disease.

Results: Forty-two patients with PAN received a total of 53 biologic courses, including TNF-α blockers in 15 cases, rituximab (RTX) in 18 cases, tocilizumab (TCZ) in 10 cases and other biologics in 10 cases.

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Background And Objectives: Spinal cord sarcoidosis is a rare manifestation of sarcoidosis with a consequent risk of neurologic sequelae for the patient. We investigated prognostic factors and efficacy of immunosuppressive treatments in a longitudinal cohort.

Methods: We retrospectively studied patients with spinal cord sarcoidosis followed between 1995 and 2021 in 7 centers in France.

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Background: Data from the PEXIVAS trial challenged the role of plasma exchange (PLEX) in ANCA-associated vasculitides (AAV). We aimed to describe kidney biopsy from patients with AAV treated with PLEX, evaluate whether histopathologic findings could predict kidney function, and identify which patients would most benefit from PLEX.

Methods: We performed a multicenter, retrospective study on 188 patients with AAV and AKI treated with PLEX and 237 not treated with PLEX.

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Tree architecture shows large genotypic variability, but how this affects water-deficit responses is poorly understood. To assess the possibility of reaching ideotypes with adequate combinations of architectural and functional traits in the face of climate change, we combined high-throughput field phenotyping and genome-wide association studies (GWAS) on an apple tree (Malus domestica) core-collection. We used terrestrial light detection and ranging (T-LiDAR) scanning and airborne multispectral and thermal imagery to monitor tree architecture, canopy shape, light interception, vegetation indices and transpiration on 241 apple cultivars submitted to progressive field soil drying.

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Genomic selection is an attractive strategy for apple breeding that could reduce the length of breeding cycles. A possible limitation to the practical implementation of this approach lies in the creation of a training set large and diverse enough to ensure accurate predictions. In this study, we investigated the potential of combining two available populations, i.

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Article Synopsis
  • The study aimed to evaluate how effective and safe off-label biologics are for patients with treatment-resistant granulomatosis with polyangiitis (GPA).
  • It involved 26 patients, most of whom received anti-TNF-α or abatacept after failing standard treatments, with most demonstrating significant disease symptoms like lung and ENT issues.
  • Results indicated that less than 50% of patients had a positive response after 6 to 12 months of treatment, with some adverse effects reported, including infections and severe reactions.
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Article Synopsis
  • The study aimed to evaluate the effectiveness and safety of rituximab and methotrexate (RTX/MTX) combination therapy in treating ANCA-associated vasculitis (AAV), specifically granulomatosis with polyangiitis (GPA).
  • A total of 17 patients with GPA participated, with most having previously failed other treatments; after 6 months, 88% showed a positive response, and by the end of the study, 94% had responded to the therapy.
  • While the combination therapy was generally well-tolerated, 41% of patients experienced severe side effects, but it was considered effective for managing persistent GPA symptoms.
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Pedigree information is of fundamental importance in breeding programs and related genetics efforts. However, many individuals have unknown pedigrees. While methods to identify and confirm direct parent-offspring relationships are routine, those for other types of close relationships have yet to be effectively and widely implemented with plants, due to complications such as asexual propagation and extensive inbreeding.

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  • The study investigates the rare association between IgA vasculitis (IgAV) and inflammatory bowel disease (IBD), primarily in patients treated with anti-TNF-α therapy.
  • Out of 43 cases analyzed, the majority showed that IBD, particularly Crohn's disease, preceded the onset of IgAV by an average of 9.2 years, with many patients experiencing purpura and joint issues.
  • The findings suggest that discontinuing anti-TNF-α can lead to remission of IgAV, but also raises the risk of IBD flare-ups, while continuing treatment may help manage IBD but lead to recurring IgAV symptoms.
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