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1. Immunovirological status in people with perinatal and adult-acquired HIV-1 infection: a multi-cohort analysis from France

Author

Aumaitre, H., Froguel, E., Caby, F., Dellion, S., Gerard, L., Lucht, F., Chirouze, C., Dupon, M., Schmit, Jl, Goujard, C., Allegre, T., Cazenave, B., Hittinger, G., De Truchis, P., Cailhol, J., Duvivier, C., Canestri, A., Bouchaud, O., Karmochkine, M., Salmon-Ceron, D., Zucman, D., Mortier, E., Tubiana, R., Girard, P.M., Pintado, C., Cabie, A., Rabier, V., Morlat, P., Neau, D., Genet, C., Makhloufi, D., Ronot, S Bregigeon, Ghosn, J., Reliquet, V., Perré, P., Pellegrin, Jl, Arvieux, C., Cheneau, C., Bernard, L., Delobel, P., Verdon, R., Jacomet, C., Piroth, L., Ajana, F., Bevilacqua, S., Debab, Y., Lecapitaine, A.L., Cotte, L., Mokhtari, S., Mercie, P., Poubeau, P., Garrait, V., Khuong, Ma, Beck-Wirth, G., Blum, L., Blanche, S., Boccara, F., Prazuck, T., Barbuat, C., Viard, J.P., Stegmann-Planchard, S., Martha, B., Treluyer, J.M., Dore, E., Gaud, C., Niault, M., Fernandes, E., Hitoto, H., Compagnucci, A., Elenga, N., Faye, A., Dollfus, C., Chace, A., Levine, M., Martha, S.A., Floch-Tudal, C., Kebaïli, K., Entz-Werle, N., Tricoire, J., Mazingue, F., Bolot, P., Brazille, P., Goetghebuer, T., Gennotte, A.F., Van Der Linden, D., Schmitz, V., Moutschen, M., Crenn-Hebert, C., Habibi, F., Coursol, A., Guesdon, E., Ceccaldi, P.F., Dehlinger – Paul, M., Pannier, E., Marcou, V., Elleau, C., Achkar, M., Vareil, M.O., Couderc, S., Routier, C., Bouldouyre, M.A., Selleret, L., Chabrol, A., Bellahcene, C., Pluchart, C., Yangui, A., Vignes, D., Alissa, A., Johnson, A., Lachassinne, E., Benbara, A., Karaoui, L., Bongain, A., Yakeu, B., Schmit, J.L., Cravello, L., Hubert, C., Faucher, P., Pinquier, D., Borie, C., Rocchi, D., Brunet-Cartier, C., Briandet, C., Brouard, J., Chalvon-Demersay, A., Rajguru, M., Billiemaz, K., Fresard, A., Moulin, A., Fialaire, P., Mesnard, L., Werner, E., Vintejoux, E., Marian, J., Ranaivojaona, S., Bissuel, F., Abdelhadi, M., Hammou, Y., Genet-Villeger, C., Hatchuel, Y., Bachelard, G., Medus, M., Dendale – Nguyen, J., Guimard, T.S., Martha, A., Rouha, M., Perfezou, P., De Saint Martin, L., Jaffuel, S., Buzele, R., Gousseff, M., Cudeville, C., Vitrat, V., Michau, C., Palenzuela, G., Driessen, M., Heller-Roussin, B., Labaune, J.M., Muanza, B., Massardier, J., Partisani, M., Hau, I., Runel-Belliard, C., Brehin, C., Kebaili, K., Lalande, M., Lagree, M., Lacombe, K., Molina, J.-M., Reynes, J., Robineau, O., Raffi, F., Becker, A., Weiss, L., Allègre, T., Pialoux, G., Souala, F., Rami, A., Katlama, C., Cabié, A., Viard, J.-P., Bastides, F., Michel, C., Salmon, D., Lièvre, J-D Le, Sotto, A., Rouveix, E., Naqvi, A., Brégigeon, S., Rodet, R., Simon-Coutelier, A., Esnault, J.-L., Buzelé, R., Stein, A., Godin-Colet, C., Pichancourt, G., Caraux-Paz, P., Zadeh, M Mohseni, Gérard, L., Lascaux-Cametz, C., Bodard, L., Pellegrin, J.-L., Ettahar, N., Uludag, A., Rosenthal, E., Prevoteau du Clary, F., Jaureguiberry, S., Philibert, P., Lecapitaine, A.-L., Chakvetadze, E., Champagne, H., Daneluzzi, V., Goupil de Bouillé, J., Leprêtre, A., Lamaury, I., Darasteanu, I., Abraham, B., Garipuy, D., Berger, J.-L., Schmit, J.-L., Diallo, K., Gourdon, F., Vaillant, O., Gaborieau, V., Doll, J., Quinsat, D., Geffray, L., Girard, J.-J., Houlbert, D., Perronne, V., Klement, E., Antioniotti, O., Rouzioux, C., Avettand-Fenoel, V., Lortholary, O., Boucly, S., Maignan, A., Thiebaut, R., Meyer, L., Boufassa, F., Charles, M.A., Dray-Spira, R., Legeai, C., Amon, V., Benammar, N., Seng, R., Slama, L., Bonnard, P., Chakvetadze, C., L’Yavanc, T., Capeau, J., Vigouroux, C., Fellahi, S., Bastard, J.P., Oksenhendler, E., Bourge, J.F., Bajzik, V., Sereni, D., Lascoux-Combe, C., Taulera, O., Dien, L.V., Delgado, J., Molina, J.M., Saint-Marc, T., Ferret, S., Pavie, J., Bergmann, J.F., Parrinello, M., BLefebvre, Boudraa, C., Diallo, B., Lupin, C., Herson, S., Simon, A., Edeb, N., Guillevin, L., Tahi, T., Pietri, M.P., Tisne-Dessus, D., Jalbert, C., Yeni, P., Matheron, S., Pahlavan, G., Phung, B., El-Alami Talbi, N., Ramani, Z., Catalano, G., Godard, C., Boue, F., Chambrin, V., Bornarel, D., Schoen, H., Carlier, R., Fantin, B., Poder, C., Dhote, R., Bentata, M., Honore, P., Tuyet, Xuan, Delfraissy, J.F., Chaix, F., Rannou, M.T., Levy, Y., Sobel, A., Dumont, C., Abel, S., Pierre-François, S., Beaujolais, V., Poizot-Martin, I., Zaegel-Faucher, O., Debreux, C., Moreau, J., Van Der Gheynst, E., Thiebaut-Drobacheff, M.C., Foltzer, A., Hoen, B., Faucher, J.F., Gil, H., Ragnaud, J.M., Raymond, I., Louis, I., Hessamfar, M., Baillat, V., De Boever, C Merle, Tramoni, C., Soufflet, A., Guadagnin, P., Choutet, P., Mounoury, O., Brosseau, D., Hue, H., May, T., Wassoumbou, S., Stenzel, M., Bouillon, M.P., Yazdanpanah, Y., Huleux, T., Aissi, E., Pavel, S., Rey, D., Fischer, P., Blaison, G., Martinot, M., Pachart, A., Jeanblanc, F., Touraine, J.L., Trepo, C., Miailhes, P., Kouadjo, K., Thoirain, V., Brochier, C., Perre, P., Leautez, S., Esnault, J.L., Suaud, I., Seng, Rémonie, Frange, Pierre, Faye, Albert, Dollfus, Catherine, le Chenadec, Jérôme, Boufassa, Faroudy, Essat, Asma, Goetghebuer, Tessa, Arezes, Elisa, Avettand-Fènoël, Véronique, Bigna, Jean-Joël, Blanche, Stéphane, Goujard, Cécile, Meyer, Laurence, Warszawski, Josiane, and Viard, Jean-Paul

Published
2024
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3. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis

Author

Emmi, G, Bettiol, A, Gelain, E, Bajema, I, Berti, A, Burns, S, Cid, M, Cohen Tervaert, J, Cottin, V, Durante, E, Holle, J, Mahr, A, Del Pero, M, Marvisi, C, Mills, J, Moiseev, S, Moosig, F, Mukhtyar, C, Neumann, T, Olivotto, I, Salvarani, C, Seeliger, B, Sinico, R, Taillé, C, Terrier, B, Venhoff, N, Bertsias, G, Guillevin, L, Jayne, D, Vaglio, A, Emmi G., Bettiol A., Gelain E., Bajema I. M., Berti A., Burns S., Cid M. C., Cohen Tervaert J. W., Cottin V., Durante E., Holle J. U., Mahr A. D., Del Pero M. M., Marvisi C., Mills J., Moiseev S., Moosig F., Mukhtyar C., Neumann T., Olivotto I., Salvarani C., Seeliger B., Sinico R. A., Taillé C., Terrier B., Venhoff N., Bertsias G., Guillevin L., Jayne D. R. W., Vaglio A., Emmi, G, Bettiol, A, Gelain, E, Bajema, I, Berti, A, Burns, S, Cid, M, Cohen Tervaert, J, Cottin, V, Durante, E, Holle, J, Mahr, A, Del Pero, M, Marvisi, C, Mills, J, Moiseev, S, Moosig, F, Mukhtyar, C, Neumann, T, Olivotto, I, Salvarani, C, Seeliger, B, Sinico, R, Taillé, C, Terrier, B, Venhoff, N, Bertsias, G, Guillevin, L, Jayne, D, Vaglio, A, Emmi G., Bettiol A., Gelain E., Bajema I. M., Berti A., Burns S., Cid M. C., Cohen Tervaert J. W., Cottin V., Durante E., Holle J. U., Mahr A. D., Del Pero M. M., Marvisi C., Mills J., Moiseev S., Moosig F., Mukhtyar C., Neumann T., Olivotto I., Salvarani C., Seeliger B., Sinico R. A., Taillé C., Terrier B., Venhoff N., Bertsias G., Guillevin L., Jayne D. R. W., and Vaglio A.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

Published
2023

5. Remission and Low Disease Activity in Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Prevalence and Impact on Damage Accrual

Author

Delvino, P, Sardanelli, F, Monti, S, Cohen, P, Puéchal, X, Mouthon, L, Montecucco, C, Guillevin, L, Terrier, B, Paolo Delvino, Federica Sardanelli, Sara Monti, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Carlomaurizio Montecucco, Loïc Guillevin, Benjamin Terrier, Delvino, P, Sardanelli, F, Monti, S, Cohen, P, Puéchal, X, Mouthon, L, Montecucco, C, Guillevin, L, Terrier, B, Paolo Delvino, Federica Sardanelli, Sara Monti, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Carlomaurizio Montecucco, Loïc Guillevin, and Benjamin Terrier

Abstract

Objective: To assess the prevalence and impact on damage accrual of different levels of disease activity in patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Methods: Patients with GPA and MPA followed for ≥5 years in 2 different centers were included. Disease activity and damage were assessed using the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI), respectively. Three levels of remission were defined: complete remission (BVAS = 0, negative for antineutrophil cytoplasmic antibody [ANCA], off treatment), clinical remission off therapy (CROffT; BVAS = 0, positive for ANCA), and clinical remission on therapy (CROnT; BVAS = 0, negative or positive for ANCA, glucocorticoids ≤5 mg/day and/or immunosuppressant). A low disease activity state (LDAS) was defined as 0 < BVAS ≤3, low-dose glucocorticoids (≤7.5 mg/day), and/or immunosuppressant. Remission or LDAS were defined as prolonged when lasting ≥2 consecutive years. Results: A total of 167 patients were included: 128 (76.6%) with GPA, 39 (23.4%) with MPA, mean ± SD age 51.0 ± 16.7 years. During a 5-year follow-up, 10 patients (6.0%) achieved prolonged complete remission, 6 (3.6%) prolonged CROffT, 89 (53.3%) prolonged CROnT, 42 (25.1%) prolonged LDAS, and 20 (12.0%) never achieved LDAS. The VDI score at 5 years progressively worsened according to increasing levels of disease activity targets (complete remission, CROffT, CROnT, and LDAS). The mean ± SD 5-year VDI score was higher in patients not achieving prolonged remission compared to those who did (3.7 ± 2.0 versus 2.2 ± 1.9; P < 0.0001). By multivariate analysis, baseline ear, nose, and throat (P = 0.006), and lung involvement (P = 0.047) were negative predictors of prolonged remission. Conclusion: More than 60% of patients with GPA/MPA achieved prolonged remission, which was associated with better long-term outcomes. In contrast, prolonged LDAS correlated with increased damage accrual and was

Published
2023

6. Séquelles à long terme au cours de la granulomatose éosinophilique avec polyangéite : données issues d’une étude rétrospective multicentrique de 845 patients

Author

Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Durel, C, Vaglio, A, Emmi, G, Guillevin, L, Terrier, B, Papo, M., Sinico, R. A., Teixeira, V., Urban, M. L., Mahrhold, J., Monti, S., Cassone, G., Schiavon, F., Seeliger, B., Neumann, T., Kroegel, C., Jayne, D., Hellmich, B., Salvarani, C., Kahn, J. E., Durel, C. A., Vaglio, A., Emmi, G., Guillevin, L., Terrier, B., Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Durel, C, Vaglio, A, Emmi, G, Guillevin, L, Terrier, B, Papo, M., Sinico, R. A., Teixeira, V., Urban, M. L., Mahrhold, J., Monti, S., Cassone, G., Schiavon, F., Seeliger, B., Neumann, T., Kroegel, C., Jayne, D., Hellmich, B., Salvarani, C., Kahn, J. E., Durel, C. A., Vaglio, A., Emmi, G., Guillevin, L., and Terrier, B.

Published
2020

10. La granulomatose éosinophilique avec polyangéite (Churg-Strauss) avec positivité des ANCA-PR3 existe-t-elle réellement ?

Author

Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Samson, M, Jayne, D, Hellmich, B, Salvarani, C, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, M., Sinico, R. A., Teixeira, V., Urban, M. L., Mahrhold, J., Monti, S., Cassone, G., Schiavon, F., Seeliger, B., Neumann, T., Kroegel, C., Groh, M., Samson, M., Jayne, D., Hellmich, B., Salvarani, C., Guillevin, L., Emmi, G., Vaglio, A., Terrier, B., Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Samson, M, Jayne, D, Hellmich, B, Salvarani, C, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, M., Sinico, R. A., Teixeira, V., Urban, M. L., Mahrhold, J., Monti, S., Cassone, G., Schiavon, F., Seeliger, B., Neumann, T., Kroegel, C., Groh, M., Samson, M., Jayne, D., Hellmich, B., Salvarani, C., Guillevin, L., Emmi, G., Vaglio, A., and Terrier, B.

Published
2019

11. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P. A., Peters, J. E., Alberici, F., Liley, J., Coulson, R. M. R., Astle, W., Baldini, C., Bonatti, F., Cid, María Cinta, Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D. R. W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M. A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G. A., Rewerska, B., Schett, George, Sinico, R. A., Szczeklik, W., Tesar, Vladimir, Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A. S., Bruce, I., Clarkson, M., Conlon, N., DasGupta, B., Doulton, T. W. R., Espígol-Frigolé, Georgina, Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernández Rodríguez, José, Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A. A., Marguerie, C., Maritati, F., Marvisi, C., McHugh, N. J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, Leonid, Pesci, Alberto, Prieto-Gonzalez, S., Ramentol-Sintas, Marc, Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans, Roser, Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R. A., Vaglio, A., Holle, J. U., Wallace, C., Smith, K. G. C., Universitat Autònoma de Barcelona, Lyons, P. A., Peters, J. E., Alberici, F., Liley, J., Coulson, R. M. R., Astle, W., Baldini, C., Bonatti, F., Cid, M. C., Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D. R. W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M. A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G. A., Rewerska, B., Schett, G., Sinico, R. A., Szczeklik, W., Tesar, V., Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A. S., Bruce, I., Clarkson, M., Conlon, N., Dasgupta, B., Doulton, T. W. R., Espigol-Frigole, G., Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernandez-Rodriguez, J., Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A. A., Marguerie, C., Maritati, F., Marvisi, C., Mchugh, N. J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, L., Pesci, A., Prieto-Gonzalez, S., Ramentol-Sintas, M., Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans-Laque, R., Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R. A., Vaglio, A., Holle, J. U., Wallace, C., Smith, K. G. C., Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, and United Kingdom Research and Innovation

Subjects
0301 basic medicine, Candidate gene, Antineutrophil Cytoplasmic, General Physics and Astronomy, Genome-wide association study, Autoimmunity, Genome-wide association studies, 0302 clinical medicine, Rheumatic diseases, immune system diseases, Eosinophilic, Eosinophilia, lcsh:Science, education.field_of_study, Multidisciplinary, Genome-wide association, eosinophilic granulomatosis with polyangiitis, ANCA status, 3. Good health, medicine.symptom, Vasculitis, Granulomatosis with polyangiitis, Antibodies, Antineutrophil Cytoplasmic, Eosinophils, Genetic Association Studies, Granulomatosis with Polyangiitis, Humans, Mendelian Randomization Analysis, Genetic Loci, Genetic Predisposition to Disease, Genome-Wide Association Study, European Vasculitis Genetics Consortium, Science, Population, General Biochemistry, Genetics and Molecular Biology, Article, Antibodies, 03 medical and health sciences, medicine, Immunogenetics, education, Rheumatology and Autoimmunity, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, Reumatologi och inflammation, business.industry, General Chemistry, medicine.disease, respiratory tract diseases, 030104 developmental biology, Immunology, EGPA, HLA, ANCA, genetics, lcsh:Q, business
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA., Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disorder characterised by asthma, eosinophilia and vasculitis. Here, the authors describe a genome-wide association study of EGPA that reveals clinical and genetic differences between subgroups stratified by autoantibody status (ANCA).

Published
2019

12. Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study

Author

Canzian A, Venhoff N, Urban ML, Sartorelli S, Ruppert AM, Groh M, Girszyn N, Taillé C, Maurier F, Cottin V, de Moreuil C, Germain V, Samson M, Jachiet M, Denis L, Rieu V, Smets P, Pugnet G, Deroux A, Durel CA, Aouba A, Cathébras P, Deligny C, Faguer S, Gil H, Godeau B, Lifermann F, Phin-Huynh S, Ruivard M, Bonniaud P, Puéchal X, Kahn JE, Thiel J, Dagna L, Guillevin L, Vaglio A, Emmi G, Terrier B, French Vasculitis Study Group (FVSG), the European EGPA Study Group., Canzian, A, Venhoff, N, Urban, Ml, Sartorelli, S, Ruppert, Am, Groh, M, Girszyn, N, Taillé, C, Maurier, F, Cottin, V, de Moreuil, C, Germain, V, Samson, M, Jachiet, M, Denis, L, Rieu, V, Smets, P, Pugnet, G, Deroux, A, Durel, Ca, Aouba, A, Cathébras, P, Deligny, C, Faguer, S, Gil, H, Godeau, B, Lifermann, F, Phin-Huynh, S, Ruivard, M, Bonniaud, P, Puéchal, X, Kahn, Je, Thiel, J, Dagna, L, Guillevin, L, Vaglio, A, Emmi, G, Terrier, B, and French Vasculitis Study Group (FVSG), the European EGPA Study Group.

Subjects
Adult, Male, medicine.medical_specialty, Immunology, Birmingham Vasculitis Activity Score, Omalizumab, Churg-Strauss Syndrome, Antibodies, Monoclonal, Humanized, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Interquartile range, Recurrence, Internal medicine, medicine, Immunology and Allergy, Humans, Immunologic Factors, 030212 general & internal medicine, Treatment Failure, Adverse effect, Glucocorticoids, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Biological Products, business.industry, Middle Aged, medicine.disease, Asthma, Treatment Outcome, Rituximab, Female, Vasculitis, business, Granulomatosis with polyangiitis, Mepolizumab, medicine.drug
Abstract

OBJECTIVE To describe the efficacy and safety of biologics for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). METHODS A retrospective European collaborative study was conducted in patients with EGPA who received treatment with biologics for refractory and/or relapsing disease. RESULTS Among the 147 patients with EGPA included in the study, 63 received rituximab (RTX), 51 received mepolizumab (MEPO), and 33 received omalizumab (OMA). At the time of inclusion, the median Birmingham Vasculitis Activity Score (BVAS) was 8.5 (interquartile range [IQR] 5-13) in the RTX group, while the median BVAS in the OMA group was 2 (IQR 1-4.5) and the median BVAS in the MEPO group was 2 (IQR 1-5). In patients receiving RTX, the median BVAS declined both at 6 months (median 1, IQR 0-4.5) and at 12 months (median 0, IQR 0-2), and the frequency of remission, partial response, treatment failure, and stopping treatment due to adverse events was 49%, 24%, 24%, and 3%, respectively. For the treatment of glucocorticoid (GC)-dependent asthma, patients who received MEPO had a much better GC-sparing effect and overall response than did patients who received OMA. The frequency of remission, partial response, treatment failure, and stopping treatment due to adverse events was 15%, 33%, 48%, and 4%, respectively, in the OMA group and 78%, 10%, 8%, and 4%, respectively, in the MEPO group. Remission rates at 12 months were 76% and 82% among patients receiving MEPO at a doses of 100 mg and 300 mg, respectively. CONCLUSION These results suggest that RTX could be effective in treating relapses of EGPA vasculitis. MEPO is highly effective with a good safety profile in patients with GC-dependent asthma. Our data suggest that 100 mg MEPO monthly could be an acceptable dosage for first-line therapy in selected instances of EGPA, recognizing, however, that this has not been compared to the validated dosage of 300 mg monthly.

Published
2020

13. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Papo, M, Sinico, R, Teixeira, V, Venhoff, N, Urban, M, Iudici, M, Mahrhold, J, Locatelli, F, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Troilo, A, Thiel, J, Hellmich, B, Monti, S, Montecucco, C, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato A, Teixeira, Vítor, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, Kroegel, Claus, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Troilo, Arianna, Thiel, Jens, Hellmich, Bernhard, Monti, Sara, Montecucco, Carlomaurizio, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Terrier, Benjamin, Papo, M, Sinico, R, Teixeira, V, Venhoff, N, Urban, M, Iudici, M, Mahrhold, J, Locatelli, F, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Troilo, A, Thiel, J, Hellmich, B, Monti, S, Montecucco, C, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato A, Teixeira, Vítor, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, Kroegel, Claus, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Troilo, Arianna, Thiel, Jens, Hellmich, Bernhard, Monti, Sara, Montecucco, Carlomaurizio, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, and Terrier, Benjamin

Abstract

OBJECTIVES: Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA. METHODS: We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status. RESULTS: ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients. CONCLUSION: PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.

Published
2021

15. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis

Author

Walsh, M, Merkel, PA, Peh, C-A, Szpirt, WM, Puéchal, X, Fujimoto, S, Hawley, CM, Khalidi, N, Floßmann, O, Wald, R, Girard, LP, Levin, A, Gregorini, G, Harper, L, Clark, WF, Pagnoux, C, Specks, U, Smyth, L, Tesar, V, Ito-Ihara, T, de Zoysa, JR, Szczeklik, W, Flores-Suárez, LF, Carette, S, Guillevin, L, Pusey, CD, Casian, AL, Brezina, B, Mazzetti, A, McAlear, CA, Broadhurst, E, Reidlinger, D, Mehta, S, Ives, N, Jayne, DRW, Walsh, M, Merkel, PA, Peh, C-A, Szpirt, WM, Puéchal, X, Fujimoto, S, Hawley, CM, Khalidi, N, Floßmann, O, Wald, R, Girard, LP, Levin, A, Gregorini, G, Harper, L, Clark, WF, Pagnoux, C, Specks, U, Smyth, L, Tesar, V, Ito-Ihara, T, de Zoysa, JR, Szczeklik, W, Flores-Suárez, LF, Carette, S, Guillevin, L, Pusey, CD, Casian, AL, Brezina, B, Mazzetti, A, McAlear, CA, Broadhurst, E, Reidlinger, D, Mehta, S, Ives, N, and Jayne, DRW

Abstract

Death from any cause or ESKD occurred in 100 of 352 patients (28.4%) in the plasma-exchange group and in 109 of 352 patients (31.0%) in the control group (hazard ratio, 0.86; 95% confidence interval [CI], 0.65 to 1.13; P=0.27). The results were similar in subgroup analyses and in analyses of secondary outcomes. We also assessed the noninferiority of a reduced-dose regimen of glucocorticoids to a standard-dose regimen, using a noninferiority margin of 11 percentage points. Death from any cause or ESKD occurred in 92 of 330 patients (27.9%) in the reduced-dose group and in 83 of 325 patients (25.5%) in the standard-dose group (absolute risk difference, 2.3 percentage points; 90% CI, −3.4 to 8.0), which met the criterion for noninferiority. Serious infections at 1 year were less common in the reduced-dose group than in the standard-dose group (incidence rate ratio, 0.69; 95% CI, 0.52 to 0.93), but other secondary outcomes were similar in the two groups.

Published
2020

16. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

Author

Jennette, J. C., Falk, R. J., Bacon, P. A., Basu, N., Cid, M. C., Ferrario, F., Flores-Suarez, L. F., Gross, W. L., Guillevin, L., Hagen, E. C., Hoffman, G. S., Jayne, D. R., Kallenberg, C. G. M., Lamprecht, P., Langford, C. A., Luqmani, R. A., Mahr, A. D., Matteson, E. L., Merkel, P. A., Ozen, S., Pusey, C. D., Rasmussen, N., Rees, A. J., Scott, D. G. I., Specks, U., Stone, J. H., Takahashi, K., and Watts, R. A.

Published
2013
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27. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P.A., Peters, J.E., Alberici, F., Liley, J., Coulson, R.M.R., Astle, W., Baldini, C., Bonatti, F., Cid, M.C., Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D.R.W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M.A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G.A., Rewerska, B., Schett, G., Sinico, R.A., Szczeklik, W., Tesar, V., Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A.S., Bruce, I., Clarkson, M., Conlon, N., DasGupta, B., Doulton, T.W.R., Espígol-Frigolé, Georgina, Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernández Rodríguez, José, Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A.A., Marguerie, C., Maritati, F., Marvisi, C., McHugh, N.J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, L., Pesci, A., Prieto-Gonzalez, S., Ramentol-Sintas, Marc, Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans-Laque, R., Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R.A., Vaglio, A., Holle, J.U., Wallace, C., Smith, K.G.C., and Universitat Autònoma de Barcelona

Subjects
Eosinophils, Genetic Loci, Granulomatosis with Polyangiitis, Humans, Genetic Predisposition to Disease, Mendelian Randomization Analysis, Genetic Association Studies, Antibodies, Antineutrophil Cytoplasmic, Genome-Wide Association Study
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

33. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, Lyons, PA, Peters, JE, Cid, MC, Little, MA, Ramierez, GA, Butterworth, AS, DasGupta, B, Doulton, TWR, Manfredi, AA, McHugh, NJ, Watts, RA, Holle, JU, Smith, KGC, Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, Lyons, PA, Peters, JE, Cid, MC, Little, MA, Ramierez, GA, Butterworth, AS, DasGupta, B, Doulton, TWR, Manfredi, AA, McHugh, NJ, Watts, RA, Holle, JU, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

34. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, Smith, KGC, Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

35. 117. Prediction of Long-Term Evolutionary Profiles in Eosinophilic Granulomatosis with Polyangiitis (Churg– Strauss) Based on Baseline and Follow-Up Characteristics

Author

Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Terrier, Benjamin, Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, and Terrier, Benjamin

Published
2019

36. 354. EFFICACY OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS TREATMENTS ACCORDING TO THE TYPE OF MANIFESTATIONS BASED ON ANALYSIS OF 636 PATIENTS

Author

Papo, M, Sinico, A, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Alberto, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Terrier, Benjamin, Papo, M, Sinico, A, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Alberto, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, and Terrier, Benjamin

Published
2019

46. Methotrexate (MTX) failed to control giant cell arteritis (GCA)

Author

Hoffman, G., Cid, M., Hellmann, D., Merkel, P., Guillevin, L., Dickler, H., Calabrese, L., Locker, G., Easley, K., Bedocs, N. M., Fortin, P., Schousboe, J., Sherrer, Y., Swanson, H., Walsh, B., Gross, W., Luqmani, R., Hunder, G., Sneller, M., Flynn, J., Stone, J., Ludivico, C., and Kalunian, K.

Published
2001

48. Caractéristiques au diagnostic et profils évolutifs des patients atteints de granulomatose éosinophilique avec polyangéite (Churg-Strauss) : données d’une étude rétrospective collaborative européenne

Author

Papo, M, Emmi, G, Schiavon, F, Groh, M, Samson, M, Kahn, J, Sinico, R, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, Sinico, RA, Papo, M, Emmi, G, Schiavon, F, Groh, M, Samson, M, Kahn, J, Sinico, R, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, and Sinico, RA

Published
2018

49. Efficacité des traitements utilisés chez les patients suivis pour une granulomatose éosinophilique avec polyangéite (Churg-Strauss) en fonction du profil évolutif de la maladie : données d’une étude rétrospective collaborative européenne

Author

Papo, M, Emmi, G, Schiavon, F, Groh, M, Kahn, J, Sinico, R, Samson, M, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, Sinico, RA, Papo, M, Emmi, G, Schiavon, F, Groh, M, Kahn, J, Sinico, R, Samson, M, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, and Sinico, RA

Published
2018

50. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

Author

Groh, M, Pagnoux, C, Baldini, C, Bel, E, Bottero, P, Cottin, V, Dalhoff, K, Dunogué, B, Gross, W, Holle, J, Humbert, M, Jayne, D, Jennette, J, Lazor, R, Mahr, A, Merkel, P, Mouthon, L, Sinico, R, Specks, U, Vaglio, A, Wechsler, M, Cordier, J, Guillevin, L, Guillevin, L., SINICO, RENATO ALBERTO, Groh, M, Pagnoux, C, Baldini, C, Bel, E, Bottero, P, Cottin, V, Dalhoff, K, Dunogué, B, Gross, W, Holle, J, Humbert, M, Jayne, D, Jennette, J, Lazor, R, Mahr, A, Merkel, P, Mouthon, L, Sinico, R, Specks, U, Vaglio, A, Wechsler, M, Cordier, J, Guillevin, L, Guillevin, L., and SINICO, RENATO ALBERTO

Abstract

Objective To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA). Methods The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached. Results Twenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed. Discussion These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.

Published
2015

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