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1. S2k-Leitlinie (Kurzfassung): Management der Großgefäßvaskulitiden

Author

Schirmer, J. H., Aries, P. M., Balzer, K., Berlit, P., Bley, T. A., Buttgereit, F., Czihal, M., Dechant, C., Dejaco, C., Garske, U., Henes, J., Holle, J. U., Holl-Ulrich, K., Lamprecht, P., Nölle, B., Moosig, F., Rech, J., Scheuermann, K., Schmalzing, M., Schmidt, W. A., Schneider, M., Schulze-Koops, H., Venhoff, N., Villiger, P. M., Witte, T., Zänker, M., and Hellmich, B.

Published
2020
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2. S2k-Leitlinie: Management der Großgefäßvaskulitiden

Author

Schirmer, J. H., Aries, P. M., Balzer, K., Berlit, P., Bley, T. A., Buttgereit, F., Czihal, M., Dechant, C., Dejaco, C., Garske, U., Henes, J., Holle, J. U., Holl-Ulrich, K., Lamprecht, P., Nölle, B., Moosig, F., Rech, J., Scheuermann, K., Schmalzing, M., Schmidt, W. A., Schneider, M., Schulze-Koops, H., Venhoff, N., Villiger, P. M., Witte, T., Zänker, M., and Hellmich, B.

Published
2020
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7. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study

Author

Bettiol, A., Urban, M. L., Dagna, L., Cottin, V., Franceschini, F., Del Giacco, S., Schiavon, F., Neumann, T., Lopalco, G., Novikov, P., Baldini, C., Lombardi, C., Berti, A., Alberici, F., Folci, M., Negrini, S., Sinico, R. A., Quartuccio, L., Lunardi, C., Parronchi, P., Moosig, F., Espigol-Frigole, G., Schroeder, J., Kernder, A. L., Monti, S., Silvagni, E., Crimi, C., Cinetto, F., Fraticelli, P., Roccatello, D., Vacca, A., Mohammad, A. J., Hellmich, B., Samson, M., Bargagli, E., Cohen Tervaert, J. W., Ribi, C., Fiori, D., Bello, F., Fagni, F., Moroni, L., Ramirez, G. A., Nasser, M., Marvisi, C., Toniati, P., Firinu, D., Padoan, R., Egan, A., Seeliger, B., Iannone, F., Salvarani, C., Jayne, D., Prisco, D., Vaglio, A., Emmi, G., Ahmad, K., Beccalli, M., Bonnotte, B., Bortolotti, R., Cariddi, A., Caminati, M., Cid, M. C., Deidda, M., Delvino, P., Scala, G. D., Felicetti, M., Ferro, F., Furini, F., Gelain, E., Ghirelli, G., Holle, J., Losappio, L. M., Mahr, A., Malandrino, D., Marhhold, J., Mattioli, I., Moi, L., Moiseev, S., Muratore, F., Nolasco, S., Olivieri, B., Palermo, A., Regola, F., Sander, O., Scarpa, R., Sciascia, S., Silvestri, E., Susca, N., Terrier, B., Treppo, E., Trezzi, B., Uzzo, M., Vitiello, G., Yacyshyn, E., RS: MHeNs - R3 - Neuroscience, Faculteit FHML Centraal, Bettiol, A, Urban, M, Dagna, L, Cottin, V, Franceschini, F, Del Giacco, S, Schiavon, F, Neumann, T, Lopalco, G, Novikov, P, Baldini, C, Lombardi, C, Berti, A, Alberici, F, Folci, M, Negrini, S, Sinico, R, Quartuccio, L, Lunardi, C, Parronchi, P, Moosig, F, Espígol-Frigolé, G, Schroeder, J, Kernder, A, Monti, S, Silvagni, E, Crimi, C, Cinetto, F, Fraticelli, P, Roccatello, D, Vacca, A, Mohammad, A, Hellmich, B, Samson, M, Bargagli, E, Cohen Tervaert, J, Ribi, C, Fiori, D, Bello, F, Fagni, F, Moroni, L, Ramirez, G, Nasser, M, Marvisi, C, Toniati, P, Firinu, D, Padoan, R, Egan, A, Seeliger, B, Iannone, F, Salvarani, C, Jayne, D, Prisco, D, Vaglio, A, Emmi, G, Jayne, David [0000-0002-1712-0637], and Apollo - University of Cambridge Repository

Subjects
Male, Epidemiology, Birmingham Vasculitis Activity Score, law.invention, Glucocorticoid, Randomized controlled trial, Prednisone, law, Eosinophilic, Monoclonal, Immunology and Allergy, Humanized, PLACEBO, Middle Aged, egpa mepolizumab, Treatment Outcome, SAFETY, Female, ANCA-associated Vasculitis, Biologicals, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss), Glucocorticoids, Granulomatosis with polyangiitis, ANCA-associated Vasculiti, medicine.drug, Keywords: ANCA-associated Vasculitis, Adult, medicine.medical_specialty, Immunology, Antibodies, Monoclonal, Humanized, Antibodies, Drug Administration Schedule, Eosinophilia, Granulomatosis with Polyangiitis, Humans, Retrospective Studies, Rheumatology, Internal medicine, medicine, Adverse effect, Asthma, business.industry, medicine.disease, Biological, EGPA, European EGPA Study Group, business, FOLLOW-UP, Mepolizumab
Abstract

OBJECTIVE: Mepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort. METHODS: We included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ���4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations. RESULTS: Two hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44). CONCLUSION: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.

Published
2022
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9. Association between age at disease onset of anti-neutrophil cytoplasmic antibody-associated vasculitis and clinical presentation and short-term outcomes

Author

Monti, S., Craven, A., Klersy, C., Montecucco, C., Caporali, R., Watts, R., Merkel, P. A., Luqmani, R., Achilleos, K., Adler, M., Alba, M. A., Albert, D. A., Alibaz-Oner, F., Allcoat, P., Amano, K., Amarasuriya, M., Amudala, N. A., Andrews, J., Archer, A. M., Arimura, Y., Atukorala, I., Azevedo, E., Bajad, S., Baldwin, C., Barra, L. J., Baslund, B., Basu, N., Baykal, M., Berger, C., Berglin, E., Besada, E., Bhardwaj, M., Bischof, A., Blockmans, D., Blood, J., Draibe, J. B., Brand, S., Brandao, M., Bruce, I. N., Butler, A., Calabrese, L. H., Ferrer, D. C., Carette, S., Carmona, D., Ceunen, H., Chakravarty, K., Chapman, P. T., Chocova, Z., Chung, S. A., Ci, W., Cid, M. C., Clark, T. M., Clarkson, M. R., De Jesus Contreras-Rodriguez, F., Conway, R., Cooke, K., Viros, X. C., Cordeiro, A., Costa, A., Culfear, K., Daikeler, T., Danda, D., Das, S. K., Dasgupta, B., De Castro, A. M., Dehghan, N., Devassy, R., Dhindsa, N., Diamantopoulos, A. P., Direskeneli, H., Dobashi, H., Juan, D., Durrani, M., Edelsten, C., Eifert, J., Elhayek, S., Elsideeg, S., Endo, T., Erden, A., Erer, B., Eriksson, P., Erturk, Z., Espigol-Frigole, G., Felicetti, M., Ferraro, A., Ferro, J. M., Fifi-Mah, A., Flores-Suarez, L. F., Flossmann, O., Flynn, D., Fonseca, J. E., Foot, J., Foote, M., Forbess, L., Fujimoto, S., Fukuoka, K., Furtado, C., Furuta, S., Gaffo, A. L., Gallagher, P., Gao, N., Gatenby, P., Gendi, N., Geraldes, R., Gerits, A., Gioffredi, A., Gomples, L., Goncalves, M. J., Gondo, P., Graham, A., Grainger, R., Gray, D. T., Grayson, P. C., Griffiths, L., Guo, Y., Gupta, R., Gylling, M., Hajj-Ali, R. A., Hammam, N., Harigai, M., Hartley, L., Haslett, J., Hassan, A., Hatemi, G., Hellmich, B., Henckaerts, L., Henes, J. C., Hepburn, J., Herd, V., Hess, C., Hill, C., Hinojosa-Azaola, A., Hirahashi, J., Hirano, F., Hocevar, A., Holle, J., Hollinger, N., Homma, S., Howard, T., Hoyles, R. K., Hruskova, Z., Hutcheon, G., Ignacak, M., Igney-Oertel, A., Ikeda, K., Ikegaya, N., Jagadeesh, S., Jaquith, J., Jayne, D. R. W., Jewell, T., Jones, C., Joshi, A., Kalyoncu, U., Kamall, S., Kamath, S., Lai, K. S., Kaname, S., Kanchinadham, S., Karadag, O., Karube, M., Kaszuba, M., Kaur, R., Kawakami, T., Kawashima, S., Khalidi, N., Khan, A., Kikuchi, M., Kilic, L., Kimura, M., King, M. J., Klapa, S., Klocke, R., Kobayashi, T., Kobayashi, S., Komagata, Y., Kronbichler, A., Kuczia, P., Kumar, M. S., Kurosawa, M., Lamprecht, P., Langford, C. A., Lanyon, P., Laversuch, C., Lee, S. J., Leoni, S., Li, J., Liang, K., Liang, P., Liao, H., Lee, L. A., Luqmani, R. A., Lyle, A., Macdonald, M., Mackie, S. L., Madden, L., Magliano, M., Makino, H., Makol, A., Malaiya, R., Malaviya, A., Manthri, R., Maritati, F., Da Silva, A. M., Mason, J. C., Matara, C., Matsui, K., Matteson, E. L., Mcbride, D., Mccullough, K., Mcgeoch, L., Mclaren, J., Mcmillian, C., Mendiratta, N., Menon, A., Merinopoulos, D., Merkel, P., Messier, S., Micheletti, R. G., Mills, K., Milman, N., Minoda, M., Minz, R. W., Mock, C., Mohammad, A. J., Moiseev, S., Moitinho, M., Molloy, E., Monach, P. A., Montgomery, M., Moosig, F., Moradizadeh, M., Morgan, M., Morgan, A. W., Morgan, A. -M., Muir, A., Mukhtyar, C., Muller, A., Muratore, F., Muso, E., Nada, R., Nakajima, H., Nakajima, T., Nakano, H., Nandagudi, A., Neumann, T., Y. F., Ng, K. H., Ng, Nogueira, E. L., Nolkha, N., Nordstrom, D., Novikov, P., Nugaliyadde, A., O'Donnell, J. L., O'Donoghue, J., O'Neill, L., O'Riordan, E., Oatley, M., Okubo, K., Oliva, E., Oshikawa, H., Ota, Y., Padoan, R., Pagnoux, C., Pan, L., Panaritis, K., Park, J. K., Patel, S., Patil, P., Pazzola, G., Peall, A., Pearce, F., Pehlevan, S., Pereira, L., Pettersson, T., Pineau, C. A., Pirila, L., Poglodek, B., Ponte, C., Prieto-Gonzalez, S., Priya, S. R., Purewal, B., Purschke, S., Putaala, J., Quickert, S., Quincey, V., Raghuvanshi, S., Rajasekhar, L., Ranganathan, D., Rathi, M., Rees, D., Rees, F., Renken, U., Restuccia, G., Rhee, R. L., Rice, B., Robins, D., Robson, J., Rodrigues, M., Romao, V. C., Rotar, C., Ruediger, C., Rutgers, A., A. C., Sa, Saavedra, M. J., Sada, K. -E., Sahbudin, I., Salvarani, C., Sandhu, N., Santos, E., Sato, Y., Schafer, V. S., Schiavon, F., Schmidt, W. A., Segelmark, M., Shahin, A., Sharma, A., Shotton, J., Silva, C., Singer, O. G., Sivasuthan, G., Smolen, S., Solanich-Moreno, X., Boixader, L. S., Song, Y. W., Springer, J., Sreih, A. G., Srivastava, R., Stamp, L. K., Stevens, R., Strbian, D., Sugino, K., Sunderkotter, C., Suppiah, R., Suzuki, K., Szekanecz, Z., Sznajd, J., Taimen, K., Tak, P. P., Takeuchi, T., Takizawa, N., Tames, L., Tan, B. E., Tanaka, M., Tang, M. W., Tatlisumak, T., Tesar, V., Thomas, A., Tian, X., Tokunaga, K., Tombetti, E., Tomsic, M., Toz, B., Tsukamoto, T., Uchida, S., Unal, A. U., Urban, M. L., Usui, J., Vaglio, A., Venkatachalam, S., Vermaak, E., Viswanath, V., Wada, T., Wagh, S., Wallace, D. J., Walters, G., Walz, B., Wan, J., Wang, T., Wang, G., Warrington, K. J., Watts, R. A., Wawrzycka-Adamczyk, K., Weeratunga, P., Weisman, M. H., Wickramasinghe, S., Williams, M., Wojcik, K., Woodruff, L., Xenitidis, T., Yamada, H., Yamagata, K., Yee, C. -S., Yoon, M., Yoshida, K., Yoshifuji, H., Ytterberg, S. R., Yumura, W., Zayed, H., Zeng, X., Zhao, M. -H., Zugaj, A., Zuk, J., İç Hastalıkları, Clinical Haematology, and Translational Immunology Groningen (TRIGR)

Subjects
Male, Outcome, Antineutrophil Cytoplasmic, 030232 urology & nephrology, 0302 clinical medicine, Risk Factors, 80 and over, Pharmacology (medical), Age of Onset, Young adult, Aged, 80 and over, education.field_of_study, age, anti-neutrophil cytoplasmic antibody-associated vasculitis, outcome, Adolescent, Adult, Aged, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Antineutrophil Cytoplasmic, Female, Humans, Middle Aged, Morbidity, Prognosis, Retrospective Studies, Risk Assessment, Survival Rate, United Kingdom, Young Adult, Vasculitis, Systemic vasculitis, medicine.medical_specialty, Population, anti-neutrophil cytoplasmic antibody–associated vasculitis, Antibodies, 03 medical and health sciences, Rheumatology, Internal medicine, medicine, education, Anti-neutrophil cytoplasmic antibody–associated vasculitis, Survival rate, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, business.industry, Retrospective cohort study, medicine.disease, Age of onset, business
Abstract

Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: Results A total of 1338 patients with AAV were included: 66% had disease onset at Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

Published
2021

15. 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

18. How I treat …

Author

Moosig, F., Reinhold-Keller, E., Holl-Ulrich, K., Feller, A. C., Bley, T., Holle, J.U., Zwerina, J., Lamprecht, P., Dalhoff, K., Venhoff, N., Thiel, J., Peter, H.-H., Laudien, M., Quetz, J., Ambrosch, P., Both, M., and Heller, M.

Published
2012
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20. [S2k guidelines: management of large-vessel vasculitis]

Author

Schirmer, J H, Aries, P M, Balzer, K, Berlit, P, Bley, T A, Buttgereit, F, Czihal, M, Dechant, C, Dejaco, C, Garske, U, Henes, J, Holle, J U, Holl-Ulrich, K, Lamprecht, P, Nölle, B, Moosig, F, Rech, J, Scheuermann, K, Schmalzing, M, Schmidt, W A, Schneider, M, Schulze-Koops, H, Venhoff, N, Villiger, P M, Witte, T, Zänker, M, and Hellmich, B

Subjects
Vasculitis, Humans, 610 Medicine & health
Published
2020

25. Influence of the IL17A locus in giant cell arteritis susceptibility

Author

Márquez, A, Hernández-Rodríguez, J, Cid, M C, Solans, R, Castañeda, S, Fernández-Contreras, M E, Ramentol, M, Morado, I C, Narváez, J, Gómez-Vaquero, C, Martínez-Taboada, V M, Ortego-Centeno, N, Sopeña, B, Monfort, J, García-Villanueva, M J, Caminal-Montero, L, de Miguel, E, Blanco, R, Palm, O, Molberg, O, Latus, J, Braun, N, Moosig, F, Witte, T, Beretta, L, Santaniello, A, Pazzola, G, Boiardi, L, Salvarani, C, González-Gay, M A, and Martín, J

Published
2014
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33. Identification of the PTPN22 functional variant R620W as susceptibility genetic factor for giant cell arteritis

Author

Serrano, A, Márquez, A, Mackie, S L, Carmona, F D, Solans, R, Miranda-Filloy, J A, Hernández-Rodríguez, J, Cid, M C, Castañeda, S, Morado, IC, Narváez, J, Blanco, R, Sopeña, B, García-Villanueva, M J, Monfort, J, Ortego-Centeno, N, Unzurrunzaga, A, Marí-Alfonso, B, Sánchez-Martín, J, de Miguel, E, Magro, C, Raya, E, Braun, N, Latus, J, Molberg, O, Lie, B A, Moosig, F, Witte, T, Morgan, A W, González-Gay, M A, and Martín, J

Published
2013
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34. International consensus on antineutrophil cytoplasm antibodies testing in eosinophilic granulomatosis with polyangiitis

Author

Moiseev, S. Bossuyt, X. Arimura, Y. Blockmans, D. Csernok, E. Damoiseaux, J. Emmi, G. Flores-Suarez, L.F. Hellmich, B. Jayne, D. Charles Jennette, J. Little, M.A. Mohammad, A.J. Moosig, F. Novikov, P. Pagnoux, C. Radice, A. Sada, K.-e. Segelmark, M. Shoenfeld, Y. Sinico, R.A. Specks, U. Terrier, B. Tzioufas, A.G. Vaglio, A. Zhao, M.-H. Tervaert, J.C.

Subjects
immune system diseases, cardiovascular diseases, urologic and male genital diseases, skin and connective tissue diseases, respiratory tract diseases
Abstract

An international consensus on antineutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosiswith polyangiitis (EGPA) is presented.ANCA, specific formyeloperoxidase (MPO), can be detected in 30-35% of patients with EGPA. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms; purpura; polyneuropathy; unexplained heart, gastrointestinal, or kidney disease; and/or pulmonary infiltrates or hemorrhage.Apositive MPO-ANCA result contributes to the diagnostic workup for EGPA. Patients with MPO-ANCA-associated EGPA have vasculitis features, such as glomerulonephritis, neuropathy, and skin manifestations, more frequently than patients with ANCA-negative EGPA. However, the presence of MPO-ANCA is neither sensitive nor specific enough to identifywhether a patient should be subclassified as having "vasculitic"or "eosinophilic"EGPA. At present, ANCA status cannot guide treatment decisions, that is,whether cyclophosphamide, rituximab, ormepolizumab should be added to conventional glucocorticoid treatment. In EGPA, monitoring of ANCA is only useful when MPO-ANCA was tested positive at disease onset. © 2020 by the American Thoracic Society.

Published
2020

41. OP0148 MEPOLIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA): A RETROSPECTIVE REAL-WORLD EUROPEAN STUDY ON 142 PATIENTS

Author

Bettiol, A., primary, Urban, M. L., additional, Alberici, F., additional, Agostini, C., additional, Baldini, C., additional, Bozzolo, E., additional, Cameli, P., additional, Crimi, N., additional, Del Giacco, S., additional, Egan, A., additional, Espigol-Frigole, G., additional, Felicetti, M., additional, Folci, M., additional, Fraticelli, P., additional, Govoni, M., additional, Kernder, A., additional, Lombardi, C., additional, Lopalco, G., additional, Lunardi, C., additional, Mohammad, A. J., additional, Moosig, F., additional, Negrini, S., additional, Neumann, T., additional, Novikov, P., additional, Paolazzi, G., additional, Parronchi, P., additional, Quartuccio, L., additional, Racanelli, V., additional, Salvarani, C., additional, Samson, M., additional, Schroeder, J., additional, Sciascia, S., additional, Sinico, R. A., additional, Terrier, B., additional, Toniati, P., additional, Prisco, D., additional, Vaglio, A., additional, and Emmi, G., additional

Published
2020
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44. MEPOLIZUMAB FOR THE TREATMENT OF PATIENTS WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A PHASE III RANDOMISED, PLACEBO-CONTROLLED TRIAL

Author

Wechsler, ME, Akuthota, P, Jayne, D, Khoury, P, Klion, A, Langford, C, Merkel, PA, Moosig, F, Specks, U, Cid, MC, Luqmani, R, Brown, J, Mallett, S, Philipson, R, Yancey, SW, Steinfeld, J, Weller, PF, and Gleich, G

Subjects
Clinical Research, 6.1 Pharmaceuticals, Clinical Trials and Supportive Activities, 32 Biomedical and Clinical Sciences, 3202 Clinical Sciences, 6 Evaluation of treatments and therapeutic interventions
Abstract

MEPOLIZUMAB FOR THE TREATMENT OF PATIENTS WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A PHASE III RANDOMISED, PLACEBO-CONTROLLED TRIAL

Published
2019
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45. Mepolizumab For The Treatment Of Patients With Eosinophilic Granulomatosis With Polyangiitis: A Phase Iii Randomized, Placebo-Controlled Trial

Author

Wechsler, M, Akuthota, P, Jayne, D, Khoury, P, Klion, A, Langford, C, Merkel, P, Moosig, F, Specks, U, Cid, M, Luqmani, R, Brown, J, Mallett, S, Philipson, R, Yancey, S, Steinfeld, J, Weller, P, and Gleich, G

Abstract

Mepolizumab For The Treatment Of Patients With Eosinophilic Granulomatosis With Polyangiitis: A Phase Iii Randomized, Placebo-Controlled Trial

Published
2019

46. Verbesserung des HbA1c bei RA Patienten mit Diabetes unter Tocilizumab

Author

Specker, C, Alberding, A, Aringer, M, Burmester, GR, Flacke, JP, Hofmann, MW, Kästner, P, Kellner, H, Moosig, F, Sieburg, M, Tony, HP, and Fliedner, G

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Einleitung: Interleukin 6 (IL-6) oder C-reaktives Protein (CRP) sind unabhängige Risikofaktoren für Typ 2 Diabetes Mellitus [ref:1] und IL-6 ist an Insulinresistenz beteiligt [ref:2]. Ogata zeigte, dass Tocilizumab (TCZ) bei diabetischen Patienten mit rheumatoider Arthritis[zum vollständigen Text gelangen Sie über die oben angegebene URL], 46. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 32. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2019
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47. 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

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