WorldCat Identities

Puéchal, Xavier

Overview
Works: 27 works in 28 publications in 2 languages and 35 library holdings
Genres: Handbooks and manuals  Case studies  Examinations 
Roles: Thesis advisor, Contributor, Other, Author
Classifications: RC927, 616.7
Publication Timeline
.
Most widely held works by Xavier Puéchal
La maladie de Whipple : à partir de quatre observations et revue de la littérature by Corinne Jubault-Adet( Book )

1 edition published in 2002 in French and held by 3 WorldCat member libraries worldwide

Inhibition of anti-tuberculosis T-lymphocyte function with tumour necrosis factor antagonists by RATIO (Recherche sur Anti-TNF et Infections Opportunistes) Study Group( )

1 edition published in 2006 in English and held by 2 WorldCat member libraries worldwide

Isolated vasculitis of the female genital tract: a case series and review of literature by Emmanuel Hoppé( )

1 edition published in 2007 in English and held by 2 WorldCat member libraries worldwide

CTLA-4+49A/G and CT60 gene polymorphisms in primary Sjögren syndrome by Jacques-Eric Gottenberg( )

1 edition published in 2007 in English and held by 2 WorldCat member libraries worldwide

Outcomes of patients admitted to intensive care units for acute manifestation of small-vessel vasculitis: a multicenter, retrospective study by Antoine Kimmoun( )

1 edition published in 2016 in English and held by 2 WorldCat member libraries worldwide

Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis by Michele Iudici( )

1 edition published in 2016 in English and held by 2 WorldCat member libraries worldwide

Contributions à l'évaluation diagnostique, pronostique et thérapeutique des vascularites systéliques by Xavier Puéchal( Book )

2 editions published in 2004 in French and held by 2 WorldCat member libraries worldwide

Au cours de la polyarthrite rhumatoïde, le spectre des vascularites est large allant d'une atteinte des capillaires à celle de l'aorte ou de ses branches.Rentabilité des biopsies musculaires contribuant à une meilleure classification des l'extension de la maladie.La maladie de Horton nécessite la réalisation d'une biopsie de l'artère temporale pour confirmer le diagnostic. La prédilection pour le territoire carotidien externe a conduit à évaluer le Doppler des axes vasculaires dans ce territoire.D'autres vascularites comme la maladie de Buerger connaissent un retard diagnostique surtout en raison d'une mauvaise connaissance des signes cliniques initiaux.Nous rapportons la 1ère série de manifestations articulaires dans une large cohorte de patients atteints de cette vascularite.Importance de la nécessité de rechercher une maladie de Buerger au cours de tte évaluation d'un rhumatisme intermittent, la présentation articulaire précédant le diagnostic de la vascularite en moyenne d'une dizaine d'années.L'expérience des vascularites considérées comme localisées au système nerveux périphérique lors du diagnostic permet d'illustrer le caractère infra-clinique de bcp d'atteintes et l'évolution vers une forme systémique chez un tiers des patients de notre cohorte.Sur le plan thérapeutique, nous rapportons les résultats d'un essai prospectif français multicentrique, coordonné par le Groupe français d'études des vascularites, dans la péri-artérite ou la micropolyangéite de mauvais pronostic.Cette étude montre que 12 bolus de cyclophosphamide exposent à moins de rechutes que 6 bolus au cours de la maladie de Horton.Le fort taux de complications iatrogènes de la corticothérapie incite à rechercher des médicaments à visée d'épargne des corticoïdes.Ainsi nous présentons une étude prospective multinationale randomisée en double aveugle, coordonnée par le Réseau international pour l'étude des vascularites, qui a comparé le méthotrexate et le placebo dans cette indication.Selon les modalités utilisées dans cette étude, le méthotrexate n'a pas montré d'effet d'épargne des corticoïdes.Nous soulignons des pistes de recherche pour l'étude de ces vascularites afin de continuer à progresser dans leur prise en charge
Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis( )

1 edition published in 2016 in English and held by 1 WorldCat member library worldwide

Abstract : Abstract: A few reports suggest combination of ANCA-associated vasculitis (AAV) and neutrophilic dermatoses (ND). We aimed to describe the main characteristics of patients presenting with both AAV and ND in a French cohort and through a systematic literature review, and to discuss the possible common pathogenic process involved. We conducted a retrospective study of patients with both conditions. Patients were selected via the French Internal Medicine Society (SNFMI) and the French Vasculitis Study Group (FVSG). A literature review focusing on a combination of both conditions, concentrated only on publications with well-established diagnoses and individual detailed data. Seventeen patients diagnosed with AAV and ND were identified in this cohort. Twelve patients had granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2 with GPA and 1 with MPA). The literature review identified 33 additional patients with both conditions, including 26 with GPA. Altogether, of the 50 patients (17 from our study and 33 from the literature review), 33 (66%) patients presented with PG associated with GPA in 29 cases (89%). Corticosteroids were the first-line treatment in conjunction with an immunosuppressive agent in most cases. Outcomes were good and a total of 15 patients experienced a relapse. Patients who relapsed were more likely to have ear, nose and throat manifestation than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%) non-relapsing patients; p = 0.03)]. In our stud, the most frequent association concerned GPA and PG. ND should be considered and specifically researched within the spectrum of cutaneous manifestations observed in AAV
Long-Term Outcomes Among Participants in the WEGENT Trial of Remission-Maintenance Therapy for Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis( )

1 edition published in 2016 in English and held by 1 WorldCat member library worldwide

Abstract : Objective: Findings from the WEGENT trial and other short-term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA). This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period. Methods: Long-term outcomes in patients enrolled in the WEGENT trial were analyzed according to their randomized treatment group (AZA or MTX). Parameters at trial entry were evaluated as potential prognostic factors for death, relapse, or damage in multivariate models. Results: Data from 10 years of followup were available for 112 (88.8%) of the 126 original trial participants. The median followup time was 11.9 years (95% confidence interval [95% CI] 11.3-12.5 years). In patients receiving AZA and those receiving MTX, the 10-year overall survival rates were 75.1% (95% CI 64.8-86.9%) and 79.9% (95% CI 70.3-90.8%) (P = 0.56), respectively, and relapse-free survival rates were 26.3% (95% CI 17.3-40.1%) and 33.5% (95% CI 23.5-47.7%) (P = 0.29), respectively. No between-treatment differences were observed with regard to rates of relapse, adverse events, damage, survival without severe side effects, and survival without relapse and severe side effects. In analyses limited to the 97 patients with GPA, no between-treatment differences in survival rates were observed. The 10-year relapse-free survival rate was lower in patients with GPA than in patients with MPA. However, in the multivariate analysis, anti-proteinase 3 antineutrophil cytoplasmic antibody (ANCA) positivity, and not GPA, was retained as being independently associated with the relapse rate. Conclusion: The results of this long-term analysis confirm that AZA and MTX are comparable treatment options for maintaining remission of GPA or MPA. Despite achieving good overall survival with these treatments, relapse rates, adverse events, and damage remain matters of concern and further studies are needed to reduce their frequency in these ANCA-associated vasculitides
Anti-IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss): Data on Seventeen Patients( )

1 edition published in 2016 in English and held by 1 WorldCat member library worldwide

Abstract : Objective: To describe the efficacy and safety of omalizumab, an anti-IgE monoclonal antibody, in patients with refractory and/or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). Methods: We conducted a nationwide retrospective study including EGPA patients who received omalizumab. Response was defined as the absence of asthma and/or sinonasal exacerbations with a prednisone dosage of d".5 mg/day (complete response) or>7.5 mg/day (partial response). Results: Seventeen patients (median age 45 years) received omalizumab for severe steroid-dependent asthma (88%) and/or sinonasal involvement (18%). After a median follow-up of 22 months, 6 patients (35%) achieved a complete response, 5 patients (30%) achieved a partial response, and 6 patients (35%) had no improvement. The median Birmingham Vasculitis Activity Score decreased from 2.5 at baseline to 0.5 at 12 months. The median number of exacerbations per month decreased from 1 at baseline to 0 at 12 months, and the median forced expiratory volume in 1 second increased from 63% of the percent predicted at baseline to 85% of the percent predicted at 12 months. The median prednisone dosage decreased from 16 mg/day at baseline to 11 mg/day at 6 months and 9 mg/day at 12 months. Omalizumab was discontinued in 8 patients (47%) during follow-up, because of remission (12.5%), adverse event despite disease remission (12.5%), refractory disease (25%), or relapse (50%). Relapses included retrobulbar optic neuritis attributable to EGPA in 2 patients and severe asthma flare in 2 others. Conclusion: The results of this study suggest that omalizumab may have a corticosteroid-sparing effect in EGPA patients with asthmatic and/or sinonasal manifestations, but reducing the corticosteroid dose may also increase the risk of severe EGPA flares, which raises the question of the safety of omalizumab in patients with EGPA
Lb02the effect of reduced-dose glucocorticoids during remission induction in severe anca associated vasculitis( )

1 edition published in 2018 in English and held by 1 WorldCat member library worldwide

Pneumomédiastin compliquant une pneumopathie interstitielle au cours des dermatomyosites et polymyosites : étude de 11 cas et revue de la littérature by Benoit Le Goff( Book )

1 edition published in 2006 in French and held by 1 WorldCat member library worldwide

Le pneumomédiastin spontané est une complication rare des myopathies inflammatoires avec une prévalence estimée à 2%. Sa survenue dans un tableau de connectivite doit faire évoquer le diagnostic de dermatomyosite ou polymyosite. Cette complication est plus fréquente chez l'homme et dans les formes cliniquement amyopathiques de dermatomyosites. Une pneumopathie interstitielle est constante, jouant possiblement un rôle via la rupture d'une alvéole ou de bulles paracardiaques. C'est une complication sévère avec une mortalité de 40%. La gravité de la pneumopathie interstitielle et sa rapidité d'évolution semblent être des facteurs de mauvais pronostic faisant discuter un traitement précoce. L'indication d'un traitement immunosuppresseur est à envisager rapidement, permettant parfois une évolution favorable sans séquelle
Pituitary Involvement in Granulomatosis With Polyangiitis( )

1 edition published in 2015 in English and held by 1 WorldCat member library worldwide

Abstract Abstract Pituitary dysfunction is a rare manifestation of granulomatosis with polyangiitis (GPA) (Wegener). The main aim of this multicenter retrospective study was to describe the characteristics and outcomes of pituitary manifestations in patients with GPA included in the French Vasculitis Study Group database. Among the 819 GPA patients included in the database, 9 (1.1%) had pituitary involvement. The median age at diagnosis of GPA and pituitary involvement was 46 and 50.8 years, respectively. Pituitary involvement was present at onset of GPA in 1 case and occurred later in 8 patients after a median follow up of 58.5 months. When pituitary dysfunction occurred, 8 patients had active disease at other sites including ENT (n = 6), eye (n = 4), or central nervous system (n = 3) involvement. The most common hormonal dysfunctions were diabetes insipidus (n = 7) and hypogonadism (n = 7). Magnetic resonance imaging was abnormal in 7 patients. The most common lesions were an enlargement of the pituitary gland, thickening of the pituitary stalk, and loss of posterior hypersignal on T1-weighed images. All patients were treated with corticosteroid therapy and 8 patients received immunosuppressive agents for the pituitary involvement, including cyclophosphamide (n = 3), rituximab (n = 2), and methotrexate (n = 3). After a median follow-up of 9.2 years, GPA was in complete remission in 7 patients, but 8 patients were still under hormone replacement therapy. Among the 5 patients who had a subsequent MRI, 2 had complete resolution of pituitary lesions. By combining our study and the literature review, the frequency of hypogonadism and diabetes insipidus, among the patients with pituitary dysfunction, can be estimated at 78% and 71% respectively. Despite a high rate of systemic disease remission on maintenance therapy, 86% of the patients had persistent pituitary dysfunction. The patients who recovered from pituitary dysfunction had all been treated by cyclophosphamide. Pituitary disease in GPA occurs mostly several months or years after diagnosis. There is no correlation between hormonal, radiologic, and systemic outcome. Although immunosuppressive drugs improve the systemic disease, hormonal deficiencies usually persist. It is therefore important to shorten diagnostic delays and treat these patients early in the course of disease before irreversible damage occur
Clinical Image: Whipple's Destructive Septic Arthritis( )

1 edition published in 2017 in English and held by 1 WorldCat member library worldwide

Ciblage pharmacologique de la cathepsine C dans une nouvelle approche thérapeutique de la granulomatose avec polyangéite by Seda Seren( )

1 edition published in 2019 in French and held by 1 WorldCat member library worldwide

Neutrophil serine proteases (NSP), maturated by cathepsin C (CatC), are the major players in neutrophil-mediated tissue degradation and immune response. Proteinase 3 (PR3) is the main target antigen of anti-neutrophil cytoplasmic auto-antibodies (ANCA) in granulomatosis with polyangiitis (GPA), a systemic small-vessel vasculitis. The pharmacological targeting of NSP by proteinase inhibitors is promising approach for GPA treatment but also for all chronic inflammatory diseases involving NSP but remains unsuccessful until now. The genetic inactivation of CatC in patients with Papillon-Lefèvre syndrome is associated with almost complete elimination of NSP in blood neutrophils. In this work, we targeted CatC using a nitrile inhibitor in neutrophil generated from umbilical cord stem cells and we observed strong reductions in intracellular and membrane- bound PR3. Among the five-proCatC activating proteases, we found CatS in neutrophilic precursor cells and in mature neutrophils. Pharmacological inhibition of CatS in neutrophils generated from stem cells resulted in significant reduction of cellular NSP. The pharmacological inhibition of CatC could help eliminate the auto- antigen of GPA and constitute a novel therapeutic strategy to reduce auto-immune inflammation in this pathology. Pharmacological targeting of both CatS and CatC might help to efficient inhibition and elimination of NSP in GPA and in chronic inflammatory diseases
Risque de rechute des vascularites associées aux ANCA diagnostiquées après 75 ans by Sara Thietart( )

1 edition published in 2020 in French and held by 1 WorldCat member library worldwide

Introduction. Les vascularites associées aux ANCA (VAA) sont fréquentes chez le sujet âgé mais peu de données sont disponibles sur le risque de rechute des patients âgés atteints de VAA. L'objectif était d'évaluer le risque de rechute des VAA chez les patients dont le diagnostic a été porté après l'âge de 75 ans.Méthodes. Les données des patients d'âge > 65 ans au diagnostic de granulomatose avec polyangéite (GPA) ou polyangéite microscopique (PAM) ont été extraites à partir de la base de données du Groupe Français d'Etude des Vascularites (GFEV) et d'un appel à dossiers envoyé aux membres du GFEV. Les caractéristiques et taux de rechutes des patients d'âge > 75 ans ont été comparés à un groupe contrôle de patients d'âge 65-75 ans. Pour évaluer le risque de rechute, les modèles de Cox et de Fine-Gray ont été utilisés, en tenant compte du décès comme étant un risque compétitif de rechute.Résultats. Sur les 299 patients inclus, 219 avaient un âge > 75 ans (médiane 79 ans) et 80 ayant un âge entre 65-75 ans (médiane 70 ans). Selon les critères EMA, 155 patients avaient un diagnostic de GPA (52 %), 136 de PAM (45 %) et 8 de VAA inclassées (3 %). Les patients d'âge > 75 ans avaient un moindre risque de rechute en analyse multivariée (CSHR 0,54, IC95 % 0,33 à 0,89 ; p=0,016 avec le modèle de Cox ; SHR 0,46, IC95 % 0,29 à 0,74 ; p=0,001, avec le modèle de Fine-Gray), même en ajustant sur le type de VAA. Les patients d'âge > 75 ans avaient une probabilité moindre d'être traités en entretien par l'association d'une corticothérapie et d'un immunosuppresseur (versus corticothérapie seule) que les patients d'âge 65-75 ans (HR 0,29, IC95 % 0,12 à 0,70 ; p=0,006), même en ajustant sur le Five Factor Score 1996 ou 2011. Les patients d'âge > 75 ans, avec indication à être traités par corticothérapie + immunosuppresseur, avaient un risque de rechute moindre lorsqu'ils suivaient ce schéma thérapeutique (CSHR 0,34, IC95 % 0,17 à 0,65 ; p=0,001).Conclusion. Les patients ayant une VAA diagnostiquée après 75 ans avaient un moindre risque de rechute que les patients d'âge 65-75 ans. Les patients d'âge > 75 ans avaient une probabilité moindre d'être traités par une association de corticothérapie + immunosuppresseur en entretien que les patients d'âge 65-75 ans. Cependant, ils bénéficiaient de ce schéma thérapeutique, qui était associé à un moindre risque de rechute
Brief Report: Childhood-Onset Systemic Necrotizing Vasculitides: Long-Term Data From the French Vasculitis Study Group Registry( )

1 edition published in 2015 in English and held by 1 WorldCat member library worldwide

Abstract Objective To describe the initial features and long-term outcomes of childhood-onset small vessel and medium vessel systemic necrotizing vasculitides (SNVs), including antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) and polyarteritis nodosa (PAN). Methods Data on patients with childhood-onset SNV registered in the French Vasculitis Study Group database were reviewed for demographic characteristics, clinical, laboratory, and histologic features, and outcomes. Disease activity was assessed with the Birmingham Vasculitis Activity Score and the Paediatric Vasculitis Activity Score, and damage was scored using the Vasculitis Damage Index. Relapse and survival rates and causes of death were analyzed. Results Fifty-six patients (35 with AAV and 21 with PAN) (median age at database enrollment 14 years [range 2-17]) were included in the study. The median duration of followup was 96 months (range 1-336); two-thirds of the patients were followed up beyond 18 years of age. Six patients (11%) died, mostly of SNV-related causes. Relapse rates ranged from 33% for microscopic polyangiitis to 50% for eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and 83% for granulomatosis with polyangiitis (Wegener's), with similar rates among AAV and PAN patients (76% and 75%, respectively); neither overall survival nor relapse-free survival differed significantly between the 2 disease groups. Rates of relapse increased after 18 years of age, both among patients with AAV and among patients with PAN. At the last followup evaluation, AAV patients had more major flares and more severe accrued damage compared with PAN patients. Conclusion Despite similar relapse rates, patients with childhood-onset AAVs experienced more major flares with more cumulative damage than those with pediatric PAN. Treatments aimed at reducing the rates of mortality and relapse in this patient group need to be developed and assessed
LES NEUROPATHIES PERIPHERIQUES PAR VASCULARITE NECROSANTE DANS LA POLYARTHRITE RHUMATOIDE : ETUDE ANATOMO-CLINIQUE ET PRONOSTIQUE ; A PROPOS DE 33 OBSERVATIONS by Xavier Puéchal( Book )

1 edition published in 1991 in French and held by 1 WorldCat member library worldwide

Rhumatologie by Collège français des enseignants en rhumatologie( Book )

1 edition published in 2011 in English and held by 1 WorldCat member library worldwide

Cet ouvrage aborde d'une façon pratique et didactique la rhumatologie. Il comprend une partie théorique et une partie entraînements. La première partie est composée des sous-parties suivantes : séméiologie (clinique, biologique, radiographique élémentaire), Données fondamentales, Conduites à tenir (transversales), Parties thématiques (pathologie ostéo-articulaire, immunorhumatologie, pathologie osseuse, rachidienne, pathologies abarticulaires et syndromes polyalgiques, pathologie podologique, pathologie musculaire, gestes locaux et rhumatologie interventionnelle). Ces chapitres suivent un plan strict : définition, épidémiologie, signes d'appel clinico-biologique, diagnostic, pronostic, éléments pathogéniques, traitement, surveillance de la maladie et du traitement, les points clés, les points de débat, CAT, RMO. La deuxième partie comprend une série de 25 cas cliniques commentés. (Somabec)
Treatment of Systemic Necrotizing Vasculitides in Patients Aged Sixty-Five Years or Older: Results of a Multicenter, Open-Label, Randomized Controlled Trial of Corticosteroid and Cyclophosphamide-Based Induction Therapy1( )

1 edition published in 2015 in English and held by 1 WorldCat member library worldwide

Abstract : Objective: To investigate a new therapeutic strategy, with rapid corticosteroid dose tapering and limited cyclophosphamide (CYC) exposure, for older patients with systemic necrotizing vasculitides (SNVs; polyarteritis nodosa [PAN], granulomatosis with polyangiitis [Wegnener's] [GPA], microscopic polyangiitis [MPA], or eosinophilic GPA [Churg-Strauss] [EGPA]). Methods: A multicenter, open-label, randomized controlled trial comprising patients e"5 years old and newly diagnosed as having SNV was conducted. The experimental treatment consisted of corticosteroids for <" months and a maximum of six 500-mg fixed-dose intravenous (IV) CYC pulses, every 2-3 weeks, then maintenance azathioprine or methotrexate. The control treatment included <"6 months of corticosteroids for all patients, combined with 500 mg/m 2 IV CYC pulses, every 2-3 weeks until remission, then maintenance for all patients with GPA or MPA and for those with EGPA or PAN with a Five-Factors Score (FFS) of e". Randomization used a 1:1 ratio computer-generated list and was performed centrally with sealed opaque envelopes. The primary outcome measure was e" serious adverse event (SAE) occurring within 3 years of followup. Secondary outcome measures included remission and relapse rates. Results: Among the 108 patients randomized, 4 were excluded (early consent withdrawal or protocol violation). Mean ± SD age at diagnosis was 75.2 ± 6.3 years. Analysis at 3 years included 53 patients (21 GPA, 21 MPA, 8 EGPA, and 3 PAN) in the experimental arm and 51 patients (15 GPA, 23 MPA, 6 EGPA, and 7 PAN) in the conventional arm. In total, 32 (60%) versus 40 (78%) had e" SAE (P = 0.04), most frequently infections; 6 (11%) versus 7 (14%) failed to achieve remission (P = 0.71); 9 (17%) versus 12 (24%) died (P = 0.41); and 20 (44%) of 45 versus 12 (29%) of 41 survivors in remission experienced a relapse (P = 0.15). Conclusion: For older SNV patients, an induction regimen limiting corticosteroid exposure and with fixed low-dose IV CYC pulses reduces SAEs in comparison to conventional therapy, and does not affect the remission rate. Three-year relapse rates remain high for both arms
 
moreShow More Titles
fewerShow Fewer Titles
Audience Level
0
Audience Level
1
  General Special  
Audience level: 0.00 (from 0.00 for La maladie ... to 0.00 for La maladie ...)

Rhumatologie
Covers
Languages
English (14)

French (7)