Bibliographies thématiques / Eosinophilic granulomatosis with polyangiitis

Littérature scientifique sur le sujet « Eosinophilic granulomatosis with polyangiitis »

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Publié le 10 mars 2023

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Articles de revues sur le sujet "Eosinophilic granulomatosis with polyangiitis"

Trivioli, Giorgio, Benjamin Terrier et Augusto Vaglio. « Eosinophilic granulomatosis with polyangiitis : understanding the disease and its management ». Rheumatology 59, Supplement_3 (29 avril 2020) : iii84—iii94. http://dx.doi.org/10.1093/rheumatology/kez570.

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Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.

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Ito, Hiroyuki, Yusuke Mishima, Tsubomi Cho, Naoki Ogiwara, Yoshimasa Shinma, Masashi Yokota, Kazuya Anzai et al. « Eosinophilic Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis ». Case Reports in Gastroenterology 14, n^o 3 (11 décembre 2020) : 668–74. http://dx.doi.org/10.1159/000511863.

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We report a case of eosinophilic cholecystitis associated with eosinophilic granulomatosis with polyangiitis (EGPA) complicated by cerebral hemorrhage. A 60-year-old man presented to a local hospital with a diagnosis of acute cholecystitis, with persistent fever and epigastric pain for 2 weeks. His symptoms persisted despite 3-week hospitalization; therefore, he was transferred to our hospital for further evaluation. Laboratory investigations upon admission showed white blood cells 26,300/µL and significant eosinophilia (eosinophils 61%). Abdominal computed tomography revealed no gallbladder enlargement but a circumferentially edematous gallbladder wall. Additional blood test results were negative for antineutrophil cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies; however, immunoglobulin (Ig)G and IgE levels were high at 1,953 mg/dL and 3,040/IU/mL, respectively. He improved following endoscopic transnasal gallbladder drainage for cholecystitis and was diagnosed with EGPA and received corticosteroid and immunosuppressant combination therapy. The eosinophil count decreased immediately after treatment, and abdominal pain and numbness resolved. He returned with left-sided suboccipital hemorrhage likely attributed to EGPA 6 months after discharge. EGPA is characterized by inflammation of small blood vessels and clinically manifests with an allergic presentation of bronchial asthma, as well as renal dysfunction, interstitial pneumonia, enteritis, and cerebral hemorrhage. Few reports have described cholecystitis as a presenting symptom of EGPA. We report a rare case of such a presentation with added considerations.

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Ogurtsova, Alina Vadimovna, Ekaterina Sergeevna Kosarenko, Tayiana Valeryrvna Zuevskaya et Evgenia Nikolaevna Ivanova. « CLINICAL CASE OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS ». Scientific medical Bulletin of Ugra 33, n^o 3 (2022) : 44–50. http://dx.doi.org/10.25017/2306-1367-2022-33-3-44-50.

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Eosinophilic granulomatosis with polyangiitis (Churg – Strauss granulomatosis) is a rare autoimmune disease characterized by eosinophilic, granulomatous inflammation in combination with necrotizing vasculitis, predominantly affecting small and medium-sized vessels and most often manifested by damage to the respiratory tract. The nonspecificity of symptoms of eosinophilic granulomatosis at the onset of the disease and the similarity of symptoms with other vasculitis, systemic, infectious and neoplastic diseases makes it difficult to make a diagnosis. The paper presents a clinical case demonstrating the difficulties of differential diagnosis of eosinophilic granulomatosis with polyangiitis with other systemic diseases.

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Pavan, Matteo, Anna Agrusti, Andrea Trombetta, Serena Pastore, Alberto Tommasini, Valentina Moressa, Federico marchetti, Andrea Taddio et Alessandro Ventura. « La granulomatosi eosinofilica con poliangioite : dalla pelle al cuore ». Medico e Bambino 39, n^o 9 (9 novembre 2020) : 569–74. http://dx.doi.org/10.53126/meb39562.

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Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

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Kavanagh, F. G., W. Hasan, D. A. Smyth et J. E. Fenton. « Polyps, grommets and eosinophilic granulomatosis with polyangiitis ». Journal of Laryngology & ; Otology 132, n^o 3 (9 janvier 2018) : 236–39. http://dx.doi.org/10.1017/s0022215117002444.

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AbstractObjective:To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.Methods:A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis.Results:Sixteen patients (10 males and 6 females) were identified. The mean age of grommet insertion was 45.4 years. The mean number of grommets inserted per patient was 1.6. The mean number of nasal polypectomies was 1.7. All 16 patients had paranasal sinus abnormalities and otitis media with effusion, 14 had asthma, 9 had serological eosinophilia and 7 had extravascular eosinophilia. Nine patients met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis.Conclusion:The co-presence of nasal polyps and resistant otitis media with effusion should raise the possibility of eosinophilic granulomatosis with polyangiitis.

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Seccia, V., L. Cristofani-Mencacci, I. Dallan, S. Fortunato, M. L. Bartoli, S. Sellari-Franceschini, M. Latorre, P. L. Paggiaro et C. Baldini. « Eosinophilic granulomatosis with polyangiitis and laryngeal involvement : review of the literature and a cross-sectional prospective experience ». Journal of Laryngology & ; Otology 132, n^o 7 (11 juin 2018) : 619–23. http://dx.doi.org/10.1017/s0022215118000737.

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AbstractBackgroundEosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis show variable otorhinolaryngological involvement. Up to 14 per cent of granulomatosis with polyangiitis patients have subglottis involvement; little is known about the laryngeal involvement in eosinophilic granulomatosis with polyangiitis.MethodA literature review was conducted, together with a prospective cross-sectional analysis of 43 eosinophilic granulomatosis with polyangiitis patients. All patients underwent fibre-optic laryngoscopy with narrow-band imaging, and completed health-related questionnaires.ResultsThe literature review showed only two cases of laryngeal involvement in eosinophilic granulomatosis with polyangiitis; in our cohort, no cases of subglottis stenosis were found, but local signs of laryngeal inflammation were present in 72 per cent of cases. Of the patients, 16.2 per cent had a pathological Reflux Finding Score (of 7 or higher).ConclusionLaryngeal inflammation in eosinophilic granulomatosis with polyangiitis is frequent. It is possibly due more to local factors than to eosinophilic granulomatosis with polyangiitis itself. However, ENT evaluation is needed to rule out possible subglottis inflammation. These findings are in line with current literature and worthy of confirmation in larger cohorts.

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Ou, Changxing, Jianjuan Ma, Ning Lai, You Li, Jiaxing Xie, Xueyan Zhang et Qingling Zhang. « Severe asthma with viral infection can develop into eosinophilic granulomatosis with polyangiitis ». Rheumatology and Immunology Research 2, n^o 4 (1 décembre 2021) : 249–54. http://dx.doi.org/10.2478/rir-2021-0034.

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Abstract Asthma is common in eosinophilic granulomatosis with polyangiitis (EGPA), and the annual incidence of EGPA in patients with asthma is much higher compared with the general population, and the trigger factor for this is unknown. We report a case of a 19-year-old male with a background of severe asthma who presented with eosinophilic lung infiltration after viral infection, which progressed to clinical EGPA. The diagnosis of EGPA was supported by an initial clinical presentation of recurrent cough and wheezing accompanied by a red rash, followed by peripheral eosinophilia, a high eosinophil percentage in bronchoalveolar lavage fluid (BALF), and migratory pulmonary eosinophilic infiltrates. Lung biopsy showed blood vessels with extravascular eosinophils. The patient responded well to high-dose glucocorticoids and cyclophosphamide, and symptoms and biochemical markers improved. Our literature review identified few reports on the triggers of EGPA, which highlights that viral infection may be a risk factor for asthma that progresses to EGPA.

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Matsumoto, Yoshinori. « Eosinophilic granulomatosis with polyangiitis ». Okayama Igakkai Zasshi (Journal of Okayama Medical Association) 134, n^o 2 (1 août 2022) : 95–98. http://dx.doi.org/10.4044/joma.134.95.

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Shukla, Ratnakar, Ashwini Kandikatla, NitinJ Nadkarni et Anant Patil. « Eosinophilic granulomatosis with polyangiitis ». International Journal of Applied and Basic Medical Research 12, n^o 2 (2022) : 148. http://dx.doi.org/10.4103/ijabmr.ijabmr_528_21.

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Korman, Abraham M., et Catherine G. Chung. « Eosinophilic Granulomatosis With Polyangiitis ». JAMA Dermatology 157, n^o 6 (1 juin 2021) : 722. http://dx.doi.org/10.1001/jamadermatol.2020.2279.

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Thèses sur le sujet "Eosinophilic granulomatosis with polyangiitis"

Zhao, Yuan. « Immunology of granulomatosis with polyangiitis ». Thesis, King's College London (University of London), 2013. https://kclpure.kcl.ac.uk/portal/en/theses/immunology-of-granulomatosis-with-polyangiitis(91230752-735f-41ea-8695-f26f8b2e5c97).html.

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Granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis) is a rare and sometimes fatal systemic autoimmune disease. Anti-neutrophil cytoplasmic antibodies (ANCAs) specific for proteinase 3 (PR3) are associated with GPA. However, the pathogenesis of GPA is not yet clear. Our aim was to investigate the local autoimmune response, circulating immune modulatory cells and cells expressing the immune suppressor molecules programmed death 1 (PD-1) and its ligands in GPA. In mucosa from GPA patients, activated B cells were observed located alongside PR3 expressing cells and B cell survival factors BAFF and APRIL, which was produced by the granulomas and giant cells. B cells were proliferating and persistent in biopsies. However no evidence of B cell clones from the mucosal biopsies circulating in peripheral blood was observed in GPA. An increased frequency of circulating TFH cells and a reduced frequency of Treg cells was observed in peripheral blood from GPA patients on conventional therapies compared to healthy controls. No such difference was found in GPA patients treated with rituximab. The frequency of circulating TFH and Treg cells was found to be inversely correlated in human peripheral blood. No difference in the relative quantity of mRNA encoding PD-1 in lymphocytes and monocytes was found in GPA patients compared with healthy controls. Lower percentage of CD14+ monocytes expressing PD-1 was observed in GPA patients. Lower relative quantity of mRNA encoding PD-1 ligands PD-L1 and PD-L2 in T cells and monocytes was observed in GPA patients. In conclusion, data in this thesis identifies activated B cells alongside auto-antigens and B cell survival factors in the mucosa in GPA. A negative correlation between TFH and Treg cells is observed that implies the balance between T cell subsets and its B cell dependence are associated with disease activity in GPA. The deficiency of PD-L1 and PD-L2 mRNA in lymphocytes and monocytes may contribute to the pathogenesis of GPA.

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Götz, Paul [Verfasser]. « Manifestation der eosinophilen Granulomatose mit Polyangiitis im Kopf-Hals-Bereich / Paul Götz ». Kiel : Universitätsbibliothek Kiel, 2017. http://d-nb.info/1136319182/34.

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Halmschlag, Kirsten [Verfasser], et Nils [Akademischer Betreuer] Venhoff. « Rituximab als Induktionstherapie der eosinophilen Granulomatose mit Polyangiitis (EGPA) im Vergleich zur Standardinduktionstherapie mit Cyclophosphamid ». Freiburg : Universität, 2016. http://d-nb.info/1124004793/34.

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Tan, Ju Ann. « All-cause and cause-specific mortality risks in granulomatosis with polyangiitis ». Thesis, University of British Columbia, 2017. http://hdl.handle.net/2429/62909.

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The full abstract for this thesis is available in the body of the thesis, and will be available when the embargo expires.
Medicine, Faculty of
Experimental Medicine, Division of
Medicine, Department of
Graduate

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Mohammad, Isa H. « Predictors of disease extension and progression in patients with granulomatosis with polyangiitis (GPA) ». Thesis, University College London (University of London), 2015. http://discovery.ucl.ac.uk/1460400/.

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Granulomatosis with Polyangiitis (GPA) is a granulomatous inflammatory disease. It is generally described as a systemic disorder which can present with a localized presentation like the orbit. Orbital GPA can be the initial manifestation of GPA where over time the disease may progress and become severe, involving vital organs. This study aimed to look for biomarkers in orbital GPA biopsies that could indicate diagnosis and be a predictor for the progression of the disease. To identify GPA patients, retrospective examination of patients’ medical records, who had undergone orbital biopsies for orbital inflammatory disease (OID), over a 21 year period, was performed. Long term outcomes of these patients were studied. Further subjective and objective histology analyses were done on haemotoxylin and eosin (H&E) tissue preparations. Comparison of cellular activity in biopsies of GPA and other OID were performed. Further T cells, B cells and macrophage phenotypes and their cytokines, were investigated with immunohistochemistry (IHC). IHC cell count comparisons were performed between GPA, sarcoidosis and idiopathic inflammatory orbital diseases (IIOD) biopsies. Results showed that in patients who presented with orbital GPA with no systemic manifestations, the disease remained localised and did not progress to systemic form, over time. H&E tissue biopsies examination showed that GPA tissues had a higher cellular activity compared to OIDs. Vasculitis and necrosis were found to be independently associated with the diagnosis of orbital GPA but these features were unreliable for diagnosis as a number of the biopsies did not exhibit these features. In immunohistochemistry staining, T cells, B cells and macrophage subtypes counts were comparable between GPA, sarcoidosis and IIOD. Nonetheless cytokines IL-17, IL-23 and BAFF-receptor (BAFF-R), were found significantly more in GPA compared to sarcoidosis and IIOD. This suggests that these cytokines possibly have a role in the pathogenesis of GPA and may have diagnostic value.

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Westman, Kerstin. « Autoimmuity in glomerulonephritis serological diagnosis and clinical outcome with special reference to Wegener's granulomatosis and microscopic polyangiitis / ». Lund : Dept. of Nephrology, University Hospital, 1997. http://catalog.hathitrust.org/api/volumes/oclc/39116623.html.

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Khasnis, Atul Ashok. « A Cost Effectiveness Analysis Of Weekly Complete Blood Count Monitoring For Leukopenia In Patients With Granulomatosis with Polyangiitis (GPA) On Cyclophosphamide ». Case Western Reserve University School of Graduate Studies / OhioLINK, 2011. http://rave.ohiolink.edu/etdc/view?acc_num=case1307639641.

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Luo, Jiao [Verfasser], Gabriela [Akademischer Betreuer] Riemekasten et Christian [Akademischer Betreuer] Heeger. « The role of IL-16 in systemic sclerosis and granulomatosis with polyangiitis / Jiao Luo ; Akademische Betreuer : Gabriela Riemekasten, Christian Heeger ». Lübeck : Zentrale Hochschulbibliothek Lübeck, 2021. http://d-nb.info/1232285188/34.

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Dourado, Letícia Barbosa Kawano. « Ativação endotelial na granulomatose com poliangeíte (granulomatose de Wegener) ». Universidade de São Paulo, 2015. http://www.teses.usp.br/teses/disponiveis/5/5150/tde-11052015-141231/.

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INTRODUÇÃO: Eventos vasculares e imunológicos são centrais na patogênese da granulomatose com poliangeíte (GPA). Moléculas de adesão celular tem papel fundamental no recrutamento de células inflamatórias do sangue para os tecidos. Diferentes leitos vasculares apresentam particularidades na expressão de moléculas de adesão celular explicando talvez parte da especificidade da GPA por determinados órgãos. A elevação no nível sérico de moléculas de adesão celular e aumento da expressão destas em amostras de biópsia renal já foram demonstrados em pacientes com vasculite ANCA associada. No entanto, o fenômeno ainda não foi estudado in situ no pulmão. OBJETIVO: O objetivo deste estudo foi analisar o padrão de expressão endotelial pulmonar de três moléculas de adesão celular na GPA, in situ: molécula de adesão intercelular 1 (ICAM-1), molécula e adesão vascular 1 (VCAM-1) e E-selectina. MÉTODOS: Examinou-se a expressão endotelial de ICAM-1, VCAM-1, E-selectina usando marcação imuno-histoquímica em secções de pulmão fixadas e parafinadas de lesões de GPA (n = 8 casos, 90 secções transversais de vasos analisados). Foram também analisados controles positivos: amostras de pulmão de doença intersticial associada à esclerodermia (SScl) (n = 8 casos, 96 secções transversais de vasos analisados) e controles negativos (n = 9 casos, 90 secções transversais de vasos analisados). A quantificação imuno-histoquímica foi realizada no aumento de 400x usando a técnica de point-counting. RESULTADOS: ICAM-1: A expressão endotelial mediana de ICAM-1 esteve aumentada de forma semelhante na GPA e na SScl (81% and 73%, respectivamente; p = 0.97). A comparação com o grupo controle (26.3%) revelou diferença estatisticamente significativa entre controle e GPA (p <0.001) quanto entre controle e SScl (p = 0.017). VCAM-1: A expressão mediana de VCAM-1 esteve significativamente aumentada na GPA se comparada a SScl (79.5% vs 41.4%; p = 0.012), no entanto, a expressão endotelial de VCAM-1 nos controles também esteve moderadamente aumentada (49.8%) e não houve diferença estatística entre SScl e controles ( p = 0.549) ou entre GPA e controles (p = 0.242). E-selectina: A expressão endotelial mediana de E-selectina esteve aumentada de forma semelhante na GPA e SScl (100% e 88.2%, respectivamente; p = 0.272). A comparação com o grupo controle (13.8%) revelou diferença estatisticamente significativa entre controle e GPA (p < 0.001) e controle e SScl (p = 0.045). CONCLUSÃO: Esses achados evidenciam o fenômeno de ativação endotelial pulmonar in situ em lesões de GPA. O perfil de expressão de moléculas de adesão parece ter particularidades em diferentes doenças a exemplo da maior expressão de VCAM-1 na GPA em relação à SScl. Essas observações contribuem para o conhecimento fisiopatogênico na GPA.
INTRODUCTION: Vascular and immunologic processes are central to the pathogenesis of granulomatosis with polyangiitis (GPA). Endothelial cellular adhesion molecules have a central role in recruiting leukocytes to sites of inflammation. Moreover, different vascular beds are phenotypically and functionally distinct with regard to expression of cellular adhesion molecules. They have been shown to be elevated in sera and in renal biopsies of patients with active ANCA-associated vasculitis. Despite of that, the expression of cellular adhesion molecules has not been studied in situ in the lungs. OBJECTIVE: Within this context, the aim of this study was to analyze the in situ pulmonary endothelial immunohistochemical pattern of expression of three cellular adhesion molecules in GPA: intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1) and E-selectin . METHODS: For such, we examined formalin-fixed, paraffin-embedded sections of lung lesions of GPA (n = 8 cases, 90 transverse sections of pulmonary vessels), negative controls which were obtained from autopsies ( n = 9 cases, 90 transverse sections of pulmonary vessels analyzed) and positive controls which were cases of interstitial lung disease associated with systemic sclerosis (SScl) (n = 8 cases, 96 transverse sections of pulmonary vessels). The quantification of the immunohistochemical staining was performed at x400 magnification using the technique of point-counting, previously described. RESULTS: ICAM-1: Median endothelial expression of ICAM-1 was similarly enhanced in GPA and SScl (81% and 73%, respectively; p = 0.97). When compared to controls (26.3%), both GPA (p <0.001) and SScl (p = 0.017) had significantly higher endothelial expression of ICAM-1. VCAM-1: Median endothelial expression of VCAM-1 was significantly enhanced in GPA when compared to SScl (79.5% vs 41.4%; p = 0.012), however the endothelial expression of VCAM-1 in the controls was also moderately enhanced (49.8%) and no statistically significant difference was found between SScl and controls ( p = 0.549) or between GPA and controls (p = 0.242). E-selectin: Median endothelial expression of E-selectin was similarly enhanced in GPA and SScl (100% and 88.2%), respectively; p = 0.272. When compared to controls (13.8%), both GPA (p < 0.001) and SScl (p = 0.045) had significantly higher endothelial expression of E-selectin. CONCLUSION: These observations are evidence of in situ pulmonary endothelial activation in lesions of GPA. The profile of expression of cellular adhesion molecules seems to be particular for each disease state and timing as evidenced by the enhanced expression of VCAM-1 in GPA when compared to SScl. These observations add information to the pathogenetic knowledge of GPA

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Seren, Seda. « Ciblage pharmacologique de la cathepsine C dans une nouvelle approche thérapeutique de la granulomatose avec polyangéite ». Thesis, Tours, 2019. http://www.theses.fr/2019TOUR3301.

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Les protéases à serine du neutrophile (PSN), maturées par une protéase à cystéine nommée la cathepsine C (CatC), sont des acteurs majeurs dans la dégradation tissulaire et la réponse immunitaire médiées par les polynucléaires neutrophiles (PNN). La protéinase 3 (PR3), l’une des PSN, est l’auto-antigène majeur de la granulomatose avec polyangéite (GPA) qui est une vascularite systémique auto-immune. Le ciblage pharmacologique des PSN par des inhibiteurs est une approche intéressante pour le traitement de la GPA mais également pour toutes les maladies inflammatoires chroniques impliquant les PSN mais reste sans résultat jusqu’à maintenant. L’inactivation génétique de la CatC chez les patients atteints du syndrome de Papillon-Lefèvre est associée à une élimination presque totale des PSN. Au cours de ce travail de thèse, nous avons ciblé la CatC à l’aide d’un inhibiteur de type nitrile dans un modèle de cellules souches différenciées en PNN et nous avons obtenu une élimination presque complète de la PR3 intracellulaire et membranaire. Dans un deuxième temps, nous avons identifié la cathepsine S (CatS) comme la protéase activatrice majeure de la CatC dans les précurseurs neutrophiliques. Nous avons donc ciblé la CatS avec un inhibiteur de type nitrile pendant la différentiation des PNN ce qui a diminué significativement les taux de PSN. L’inhibition pharmacologique de la CatC pourrait donc éliminer l’auto- antigène de la GPA et constituer une nouvelle stratégie thérapeutique innovante pour contrôler l’auto-immunité et l’inflammation associée dans cette pathologie. Une combinaison d’inhibiteurs de CatS et de CatC pourrait s’avérer plus efficace pour éliminer la PR3 ainsi que les PSN pro-inflammatoires dans la GPA et dans d’autres pathologies inflammatoires chroniques
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

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Livres sur le sujet "Eosinophilic granulomatosis with polyangiitis"

Lindberg, Ronny. On granulomatous enteritis and eosinophilic granulomatosis in the horse. Uppsala : Sveriges Lantbruksuniveristet, 1985.

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L, Gross Wolfgang, International Workshop on ANCA, (4th : 1992 : Lübeck, Germany) et International Colloquium on Wegener's Granulomatosis and Vasculitic Disorders, (2nd : 1992 : Lübeck, Germany), dir. ANCA-associated vasculitides : Immunological and clinical aspects. New York : Plenum Press, 1993.

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A, Sessa, Meroni Mietta et Battini Graziana, dir. Renal involvement in systemic vasculitis. Basel : Karger, 1991.

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Gross, Wolfgang L., et Julia U. Holle. Clinical features of ANCA-associated vasculitis. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199642489.003.0131.

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The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a 'pure' small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

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Jolly, Elaine, Andrew Fry et Afzal Chaudhry, dir. Rheumatology. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199230457.003.0019.

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Chapter 19 covers the basic science and clinical topics relating to rheumatology which trainees are required to learn as part of their basic training and demonstrate in the MRCP. It covers basic science, the synovium, autoantibodies, osteoarthritis, rheumatoid arthritis, septic arthritis, crystal arthropathies, spondyloarthritides, psoriatic arthritis, low back pain, systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, Sjögren syndrome, giant cell arteritis/polymyalgia rheumatic, polyarteritis nodosa, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), granulomatosis with polyangiitis (Wegener), treating systemic vasculitis, relapsing polychondritis, and Behҫet disease.

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Jayne, David. Treatment of ANCA-associated vasculitis. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199642489.003.0132.

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The goals of treatment in anti-neutrophil cytoplasm antibody (ANCA) vasculitis are to stop vasculitic activity, to prevent vasculitis returning, and to address longer-term comorbidities caused by tissue damage, drug toxicity, and increased cardiovascular and malignancy risk. Cyclophosphamide and high-dose glucocorticoids remain the standard induction therapy with alternative immunosuppressives, such as methotrexate or azathioprine, to prevent relapse. Refractory disease resulting from a failure of induction or remission maintenance therapy requires alternative agents and rituximab has been particularly effective. Replacement of cyclophosphamide by rituximab for remission induction is supported by recent evidence. Additional therapy with intravenous methylprednisolone and plasma exchange is employed in severe presentations with failing vital organ function. Drug toxicity contributes to comorbidity and mortality and has led to newer regimens with reduced cyclophosphamide exposure. Glucocorticoid toxicity remains a major problem, with controversy over the rapidity with which glucocorticoids can be reduced or withdrawn. Disease relapse occurs in 50% and requires early detection at a stage when it will not adversely affect outcomes. Rates of cardiovascular disease and malignancy are higher than in control populations but strategies to reduce their risk, apart from cyclophosphamide-sparing regimens, have not been developed. Thromboembolic events occur in 10% and may be linked to the recently identified autoantibodies to plasminogen and tissue plasminogen activator. Outcomes of vasculitis depend heavily on the level of tissue damage at diagnosis, especially renal dysfunction, but are also influenced by patient age, ANCA subtype, disease extent, and response to therapy. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)is treated along similar principles to granulomatosis with polyangiitis (GPA) and microscopic polyangiitis but the persistence of steroid-dependent asthma in over one-third and differences in pathogenesis has suggested alternative treatment approaches. Chronic morbidity results from tissue damage and is especially common in the upper and lower respiratory tract and kidneys. Tracheobronchial disease is a severe late complication of GPA, while deafness, nasal obstruction, and chronic sinusitis are sequelae of nasal and ear vasculitis. Chronic infection of damaged epithelial surfaces acts as a drive for vasculitic activity and adequate infection control is necessary for stable remission. Chronic kidney disease can stabilize for many years but the risks of endstage renal disease (ESRD) are increased by acute kidney injury at presentation or renal relapse. Renal transplantation is successful, with similar outcomes to other causes of ESRD.

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Reid, Luke. Granulomatosis with polyangiitis. Sous la direction de John Phillips et Sally Erskine. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198834281.003.0045.

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Koelle, Daria. Coloring Book - You Will Get Better - Granulomatosis with Polyangiitis : Heal While Coloring. Independently Published, 2021.

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Sudhir, Rajini. Pulmonary vasculitis. Sous la direction de Patrick Davey et David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0140.

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Pulmonary vasculitis comprises a heterogeneous group of disorders characterized by an inflammatory process damaging the vessel wall, leading to ischaemia and tissue necrosis. Wegener’s granulomatosis, Churg–Strauss syndrome, and microscopic polyangiitis are primary, small-vessel, necrotizing vasculitides linked by an overlapping clinicopathological picture and are referred to collectively as ANCA-associated systemic vasculitis. The European Vasculitis Study Group proposed a clinical staging system based on disease activity, to guide treatment.

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Keogh, Karina A. Vasculitis in the critically ill. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199600830.003.0277.

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The vasculitic syndromes are a heterogeneous group of rare disorders characterized by degrees of inflammation and necrosis of blood vessels with a wide variety of clinical manifestations. Intensive care treatment is most commonly required for vasculitis involving small blood vessels, including capillaries. Involvement of these vessels in the lung causes alveolar haemorrhage, which may lead to respiratory failure. In the kidneys it may cause glomerulonephritis leading to renal failure. Severe cardiac, neurological, and gastrointestinal manifestions can also be seen. Non-vasculitic manifestations may also be present, such as pulmonary nodules secondary to granulomatous inflammation in granulomatosis with polyangiitis. Diagnosis is based primarily on history and physical exam in conjunction with radiographic and serological testing. Intensive care unit admission is typically secondary to end organ damage due to inflammation, or because of side effects from the cytotoxic therapies, particularly infection. Treatment of vasculitis includes supportive management in conjunction with immunosuppression. Standard treatment of severe disease consists of corticosteroids and cytotoxic drugs.

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Chapitres de livres sur le sujet "Eosinophilic granulomatosis with polyangiitis"

Nanzer, Alexandra M., et Michael E. Wechsler. « Eosinophilic granulomatosis with polyangiitis ». Dans Eosinophilic Lung Diseases, 177–92. Sheffield, United Kingdom : European Respiratory Society, 2022. http://dx.doi.org/10.1183/2312508x.10029820.

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Zakowski, M., J. Murphy, J. Ross et L. Coleman. « Eosinophilic Granulomatosis with Polyangiitis ». Dans Consults in Obstetric Anesthesiology, 189–90. Cham : Springer International Publishing, 2018. http://dx.doi.org/10.1007/978-3-319-59680-8_52.

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Cao, Yujie, et Huabin Li. « Eosinophilic Granulomatosis with Polyangiitis ». Dans Chronic Rhinosinusitis, 299–302. Singapore : Springer Singapore, 2022. http://dx.doi.org/10.1007/978-981-16-0784-4_34.

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Micheletti, Robert G. « Eosinophilic Granulomatosis with Polyangiitis ». Dans Inpatient Dermatology, 241–45. Cham : Springer International Publishing, 2018. http://dx.doi.org/10.1007/978-3-319-18449-4_49.

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Scapa, Jason V., et Gregory A. Fishbein. « Eosinophilic Granulomatosis with Polyangiitis Versus Eosinophilic Pneumonia ». Dans Practical Lung Pathology, 305–10. Cham : Springer International Publishing, 2022. http://dx.doi.org/10.1007/978-3-031-14402-8_50.

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Sinico, Renato Alberto, et Paolo Bottero. « Eosinophilic Granulomatosis with Polyangiitis (Churg-Straus Syndrome) ». Dans Systemic Vasculitides : Current Status and Perspectives, 129–39. Cham : Springer International Publishing, 2016. http://dx.doi.org/10.1007/978-3-319-40136-2_12.

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Pagnoux, Christian, et Loïc Guillevin. « Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss Syndrome) ». Dans Skin Manifestations in Rheumatic Disease, 297–303. New York, NY : Springer New York, 2013. http://dx.doi.org/10.1007/978-1-4614-7849-2_36.

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Nguyen, Yann, et Loïc Guillevin. « Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg–Strauss) ». Dans Rare Diseases of the Immune System, 77–95. Cham : Springer International Publishing, 2019. http://dx.doi.org/10.1007/978-3-030-02239-6_6.

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Vaglio, Augusto, et Lucio Manenti. « Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) ». Dans Encyclopedia of Medical Immunology, 391–99. New York, NY : Springer New York, 2014. http://dx.doi.org/10.1007/978-0-387-84828-0_343.

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Pagnoux, Christian, et Loïc Guillevin. « Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) ». Dans Inflammatory Diseases of Blood Vessels, 252–62. Oxford, UK : Wiley-Blackwell, 2012. http://dx.doi.org/10.1002/9781118355244.ch23.

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Actes de conférences sur le sujet "Eosinophilic granulomatosis with polyangiitis"

Ha, G., S. Randhawa et S. J. Evans. « Eosinophilic Pneumonia Due to Eosinophilic Granulomatosis with Polyangiitis ». Dans American Thoracic Society 2022 International Conference, May 13-18, 2022 - San Francisco, CA. American Thoracic Society, 2022. http://dx.doi.org/10.1164/ajrccm-conference.2022.205.1_meetingabstracts.a2652.

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Ullah, S., F. Nayeemuddin, P. P. Kyaw et R. Sreedhar. « Anca Negative Eosinophilic Granulomatosis with Polyangiitis ». Dans American Thoracic Society 2019 International Conference, May 17-22, 2019 - Dallas, TX. American Thoracic Society, 2019. http://dx.doi.org/10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a3109.

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PEREIRA, FLÁVIO RIBEIRO, JANNINE FARIAS BELLINI LEITE, ISABELLA MATIAS RIBEIRO, FERNANDA MARVILA FAGUNDES LAMARCA, NATÁLIA MIRANDA GAVA, NÁDIA CHRISTINA MAIA MOREIRA, INGRID CARVALHO ANDRADE et PAULA AMANDA STARLING ALVES. « EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS : CASE REPORT. » Dans 36º Congresso Brasileiro de Reumatologia. São Paulo : Editora Blucher, 2019. http://dx.doi.org/10.5151/sbr2019-096.

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Kubbara, A., E. S. Yi et M. Baqir. « A Case of Eosinophilic Granulomatosis with Polyangiitis Mimic ». Dans American Thoracic Society 2020 International Conference, May 15-20, 2020 - Philadelphia, PA. American Thoracic Society, 2020. http://dx.doi.org/10.1164/ajrccm-conference.2020.201.1_meetingabstracts.a3269.

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Rednic, D., R. Rancea, F. Cozma, L. Damian, L. Muntean, MM Tamas, S.-P. Simon, I. Felea et S. Rednic. « AB0557 Heart lesson in eosinophilic granulomatosis with polyangiitis ». Dans Annual European Congress of Rheumatology, 14–17 June, 2017. BMJ Publishing Group Ltd and European League Against Rheumatism, 2017. http://dx.doi.org/10.1136/annrheumdis-2017-eular.6383.

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de Quadros Martins, Vanessa, Jean Paulo Veronese de Souza, Guilherme Stüker, Roberta Casanova Wilhelms, Camila Schafer, Deise Marcela Piovasan, Bárbara Mendes da Silva, Marcelo Maltchik, Iloite Maria Scheibel et Markus Bredemeier. « EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS MIMICKING GUILLAIN-BARRÉ SYNDROME ». Dans Congresso Brasileiro de Reumatologia 2020. Sociedade Brasileira de Reumatologia, 2021. http://dx.doi.org/10.47660/cbr.2020.17201.

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Souza, Marília Voges de, Mauro Waldemar Keiserman, Cristina Ferreira Rabelo, Inês Guimarães Silveira, Marcel Mathias Villaça, Camila Ribeiro Dutra, José Pedro Duarte Hillal et al. « Diffuse alveolar hemorrhage in eosinophilic granulomatosis with polyangiitis ». Dans XXXIX Congresso Brasileiro de Reumatologia. Sociedade Brasileiro de Reumatologia, 2022. http://dx.doi.org/10.47660/cbr.2022.1840.

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Ward, K., A. Douglas, A. Tanna, SP McAdoo, C. Pusey et PW Ind. « P97 Rituximab treatment for eosinophilic granulomatosis with polyangiitis ». Dans British Thoracic Society Winter Meeting 2019, QEII Centre, Broad Sanctuary, Westminster, London SW1P 3EE, 4 to 6 December 2019, Programme and Abstracts. BMJ Publishing Group Ltd and British Thoracic Society, 2019. http://dx.doi.org/10.1136/thorax-2019-btsabstracts2019.240.

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Gunn, E., et M. L. Curtiss. « Eosinophilic Granulomatosis with Polyangiitis : A Presentation Rarely Seen ». Dans American Thoracic Society 2022 International Conference, May 13-18, 2022 - San Francisco, CA. American Thoracic Society, 2022. http://dx.doi.org/10.1164/ajrccm-conference.2022.205.1_meetingabstracts.a2649.

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Dipe Prates Miranda, Luiz Felipe, Maria Carolina Fonseca Coelho, Rafaela Vieralves João Izzo Pinto, Débora Rocha de Moura Rodrigues de Aguiar et Kátia Cristina Carvalho de Melo Matos. « MASTITIS REVEALING EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS : A CASE REPORT ». Dans Congresso Brasileiro de Reumatologia 2020. Sociedade Brasileira de Reumatologia, 2021. http://dx.doi.org/10.47660/cbr.2020.17452.

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