Publications by authors named "Pierre Duffau"
Chronic histiocytic intervillositis of unknown origin (CHI) is a rare placental disorder associated with adverse pregnancy outcomes, frequent recurrence, and a lack of effective preventive strategies. Recent insights indicate a potential link between CHI-associated inflammatory lesions and the inflammasome pathway, suggesting innovative therapeutic avenues. Here we show a potential role of the inflammasome pathway in CHI through comprehensive transcriptomic analysis of grade 2 or 3 histopathologic CHI samples, paired with placental controls.
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- Systemic sclerosis (SSc) is a severe disease linked to high mortality rates, primarily due to related health issues known as comorbidities, which were analyzed in this study to create a 'comorbidome.'
- The study involved 400 patients, revealing that specific conditions like cancer, heart disease, and multiple medications significantly contributed to mortality rates.
- The findings emphasized the importance of these comorbidities, although the study's small sample size suggests the need for further research to validate the results and possibly develop a mortality risk assessment tool.
In primary immune thrombocytopenia (ITP), predictors of disease evolution and treatment response are needed. Data based on the site of platelet destruction are scarce. We performed a retrospective single-centre study of adult patients with primary ITP undergoing at least one Indium-111 platelet scintigraphy (IPS) between 2009 and 2018.
View Article and Find Full Text PDFIntroduction: Neurosarcoidosis (NS) is a systemic inflammatory granulomatous disease affecting of patients with sarcoidosis. Its diagnosis is difficult as there is no specific test for it. Because of its rarity, the management of NS has so far only been described in case series and short retrospective cohorts.
View Article and Find Full Text PDFPreventing hyperhomocysteinemia using vitamin B supplementation in Givosiran-treated acute intermittent porphyria: Highlights from a case report and brief literature review.
Mol Genet Metab Rep
June 2024
Article Synopsis
- Acute hepatic porphyrias are genetic disorders that disrupt heme production, leading to harmful substances and severe neurological attacks, with givosiran being a new treatment targeting a key enzyme involved in this process.
- A case study of a 72-year-old patient showed that givosiran treatment caused high levels of homocysteine, prompting treatment discontinuation and revealing a possible connection to a deficiency in the cystathionine β-synthase enzyme.
- Ongoing vitamin B supplementation normalized homocysteine levels while maintaining givosiran treatment, highlighting the need to monitor vitamin status and homocysteine metabolism in patients receiving therapy for better management.
Background: Infliximab is currently recommended as a third-line treatment for refractory sarcoidosis. Data in function of clinical phenotype are currently lacking. We evaluated patients' characteristics and responses to infliximab according to their GenPhenReSa cluster.
View Article and Find Full Text PDFObjectives: To describe the characteristics of patients with Sjögren's disease (SjD) and inclusion-body myositis (IBM), and how they compare to SjD patients with other inflammatory myopathies (IM).
Methods: Patients were retrospectively recruited from 13 French centers and included if they met the ACR/EULAR criteria for SjD and for IM. They were categorized as SjD-IBM if sub-criteria for IBM were met, or as SjD-other IM if not.
Background: Neurosarcoidosis is a rare manifestation of sarcoidosis with heterogeneous presentations. Patient management is challenging due to the current lack of knowledge about the long-term disease course.
Objective: To identify specific disease courses of neurosarcoidosis according to the clinical and paraclinical presentations at onset.
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.
Article Synopsis
- A study assessed the quality of life (QoL) of people living with HIV in Nouvelle Aquitaine, France, revealing that while 63.5% reported good/very good QoL, significant improvements are still needed.
- The highest QoL scores were in physical and environmental domains (both median 69), while social and psychological scores were notably lower (both median 56).
- Factors like having three or more comorbidities, experiencing HIV-related stigma, and a monthly income below €1500 were linked to poorer QoL, highlighting the need to address both health and social issues to improve outcomes for all PLWH.
Article Synopsis
- The study investigated the effects of the COVID-19 pandemic on the initiation and success of antiretroviral therapy (ART) for people diagnosed with HIV in the Aquitaine region of France, comparing cases from the pandemic period (2020-2021) to those diagnosed before (2018-2020).
- It involved adults diagnosed with HIV-1 and assessed key metrics such as the time from diagnosis to ART initiation and achieved viral suppression.
- Results showed that individuals diagnosed during the pandemic had a higher likelihood of starting therapy and achieving viral suppression than those diagnosed earlier, but the study noted fewer foreign-born individuals were diagnosed during the pandemic, suggesting potential barriers to care.
Background: It is unclear whether hereditary hemorrhagic telangiectasia (HHT) patients can tolerate antithrombotic therapies (AT) including antiplatelet (AP) and/or anticoagulant (AC) agents.
Objectives: Primary endpoint was tolerance to AT in HHT. Secondary endpoints were to identify factors associated with major bleeding events (MBE) and premature discontinuation of AT.
Article Synopsis
- A study was conducted to investigate the characteristics and outcomes of ANCA-associated vasculitis (AAV) caused by anti-thyroid drugs (ATDs), specifically focusing on microscopic polyangiitis (MPA).
- The research included 45 patients with ATD-induced AAV, and it was found that most had positive ANCA results, with skin complications and joint pain being the primary symptoms.
- Compared to primary MPA, ATD-induced MPA patients were younger, had more skin problems and fewer kidney issues, and experienced a lower risk of disease relapse after treatment.
Objective: Among specific autoantibodies in DM, the anti-small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE-positive DM.
Methods: Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study.
Objective: We aimed to determine the contribution of inflammasome activation in chronic low-grade systemic inflammation observed in patients with HIV (PWH) on long-term suppressive antiretroviral therapy (ART) and to explore mechanisms of such activation.
Design: Forty-two PWH on long-term suppressive ART (HIV-RNA < 40 copies/ml) were compared with 10 HIV-negative healthy controls (HC).
Methods: Inflammasome activation was measured by dosing mature interleukin (IL)-1β and IL-18 cytokines in patient serum.
Introduction: Clinical and immunological features of patients with cancer-associated systemic sclerosis: an observational study.
Objective: Several studies have reported an increased incidence of cancer in patients with systemic sclerosis (SSc). The presence of RNA polymerase III antibodies (anti-RNA Pol 3) associates with an increased risk of cancer, but other risk factors need yet to be identified.
Bilateral adrenal hemorrhage is a rare cause of adrenal insufficiency which has been rarely associated with myeloproliferative neoplasms. Here, we report two cases of bilateral adrenal hemorrhage revealed by abdominal pain, malaise, and fatigue in two octogenarian males previously diagnosed with JAK2 V617F-positive essential thrombocythemia. Both patients were on long-term direct oral anticoagulant treatment for atrial fibrillation.
View Article and Find Full Text PDFBackground: To evaluate drug use (alcohol, tobacco, cannabis and other drugs) and its association with mean CD4/CD8 T cell count ratio, a marker of chronic inflammation, in virally suppressed people living with HIV-1 (PLWH) in Nouvelle Aquitaine, France.
Methods: A multi-centric, cross-sectional analysis was conducted in 2018-19 in the QuAliV study-ANRS CO3 AQUIVIH-NA cohort. Tobacco, alcohol, cannabis, and other drug use (poppers, cocaine, amphetamines, synthetic cathinones, GHB/GBL) were self-reported.
Background: Severe non-AIDS bacterial infections (SBIs) are among the leading causes of hospital admissions among persons with human immunodeficiency virus (PWH) in regions with high antiretroviral therapy coverage.
Methods: This large prospective cohort study of PWH examined the types of infections, bacterial documentation, and evolution of antibiotic resistance among PWH hospitalized with SBIs over an 18-year period.
Results: Between 2000 and 2017, 459 PWH had at least 1 SBI with bacterial documentation.
Importance: Catastrophic antiphospholipid syndrome (CAPS) is a severe, rare complication of antiphospholipid syndrome (APS), but cutaneous involvement has not yet been adequately described.
Objective: To describe cutaneous involvement during CAPS, its clinical and pathological features, and outcomes.
Design, Setting, And Participants: This cohort study was a retrospective analysis of patients included in the French multicenter APS/systemic lupus erythematosus register (ClinicalTrials.
Article Synopsis
Objective: Despite an important increase in lifespan over the last decades, patients with systemic lupus erythematosus (SLE) still have to face a high morbi-mortality, particularly related to cardiovascular diseases, infections and cancers. Such events are more commonly found during old age in the general population, raising the hypothesis of an acceleration of the aging process in SLE patients. In this pilot study, we wanted to test the hypothesis that SLE would be associated with an accelerated biological aging measured by the epigenetic clocks models.
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