Search

Your search keyword '"Holle, J."' showing total 764 results
Start Over Author "Holle, J."
764 results on '"Holle, J."'

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
Full Text
View/download PDF

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
Full Text
View/download PDF

4. 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. Neuralgic Amyotrophy: A Commonly Overlooked Cause of Acute Shoulder Pain.

Author

Holle, J. F., Limmroth, V., Windisch, W., and Zimmermann, M.

Abstract

Background: Neuralgic amyotrophy (NA) is a multifactorial, monophasic neuritis that mainly affects the nerves of the shoulder girdle. It is characterized by very severe pain and by weakness that arises some time after the pain. Its reported incidence is high (100 cases per 100 000 persons per year), but our data suggest that many or most cases are diagnosed late or not at all. Methods: This review of the epidemiology, pathophysiology, diagnosis, and treatment of NA is based on pertinent publications retrieved by a selective literature search, and on data provided by the scientific institute of AOK, a German statutory health-insurance carrier. Results: It is currently thought that the combination of a genetic predisposition, an immunological trigger factor, and mechanical stress on the affected nerve segment(s) is pathophysiologically determinative. The prognosis of untreated NA is poor, with 25% of patients remaining unable to work at three years. The main form of treatment is with corticosteroids that are administered as early as possible. If there is evidence of nerve constriction or torsion, surgery may also help. There have only been six controlled cohort studies on the treatment of NA, and no randomized trials. It is not uncommon for the acute phase to develop into a chronic pain syndrome requiring multidimensional treatment. Conclusion: Particularly in view of the high incidence and improved therapeutic options, NA should be included in the differential diagnosis of all patients with suggestive symptoms. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

6. Charakterisierung von Patienten mit atypischem hämolytisch-urämischen Syndrom (aHUS) in Deutschland: Daten aus dem globalen aHUS-Register

Author

Haas, C. S., Nitschke, M., Menne, J., Guthoff, M., Gäckler, A., Bruck, H., Pape, L., Vester, U., Wühl, E., Billing, H., Herbst, R., Thaiss, F., Hoppe, B., Weber, L. T., Zschiedrich, S., Feldkamp, T., Oh, J., Bald, M., Schröppel, B., Holle, J. B., Jabs, W., Beckermann, J., Budde, K., Faulhaber-Walter, R., and Schaefer, F.

Published
2019
Full Text
View/download PDF

11. OP0102 IDENTIFICATION OF NEW RISK LOCI AND PATHWAYS INVOLVED IN GCA PATHOGENESIS BY A GENOME-WIDE STUDY

Author

Borrego-Yaniz, G., primary, Ortiz-Fernández, L., additional, Kerick, M., additional, Madrid-Paredes, A., additional, Vaglio, A., additional, Hernández-Rodríguez, J., additional, Mackie, S., additional, Castañeda, S., additional, Solans-Laqué, R., additional, Mestre, J., additional, Dasgupta, B., additional, Watts, R., additional, Khalidi, N., additional, Langford, C., additional, Ytterberg, S. R., additional, Beretta, L., additional, Govoni, M., additional, Emmi, G., additional, Cimmino, M. A., additional, Witte, T., additional, Neumann, T., additional, Holle, J., additional, Schönau, V., additional, Pugnet, G., additional, Papo, T., additional, Haroche, J., additional, Mahr, A., additional, Mouthon, L., additional, Molberg, Ø., additional, Diamantopoulos, A., additional, Voskuyl, A., additional, Daikeler, T., additional, Berger, C., additional, Molloy, E., additional, Blockmans, D., additional, Consortium, U. G., additional, Gca Consortium, I., additional, Ortego, N., additional, Brouwer, E., additional, Lamprecht, P., additional, Klapa, S., additional, Salvarani, C., additional, Merkel, P. A., additional, Cid, M. C., additional, González-Gay, M. A., additional, Morgan, A., additional, Martin Ibanez, J., additional, and Márquez, A., additional

Published
2023
Full Text
View/download PDF

12. Sicherheit und Wirksamkeitshinweise zum Off-label-Einsatz von Biologikatherapien nach Versagen konventioneller Therapien bei Patienten mit entzündlich rheumatischen Erkrankungen: Ergebnisse eines nationalen Registers (GRAID2)

Author

Proft, F., Schulze-Koops, H., Grunke, M., Schrezenmeier, E., Halleck, F., Henes, J., Unger, L., Schmidt, E., Fiehn, C., Jacobi, A., Iking-Konert, C., Kneitz, C., Schmidt, R. E., Bannert, B., Voll, R. E., Fischer-Betz, R., Kötter, I., Tony, H. P., Holle, J., Aringer, M., Erler, A., Behrens, F., Burmester, G. R., and Dörner, T.

Published
2018
Full Text
View/download PDF

16. Erratum zu: Charakterisierung von Patienten mit atypischem hämolytisch-urämischen Syndrom (aHUS) in Deutschland: Daten aus dem globalen aHUS-Register

Author

Haas, C. S., Nitschke, M., Menne, J., Guthoff, M., Gäckler, A., Bruck, H., Pape, L., Vester, U., Wühl, E., Billing, H., Herbst, R., Thaiss, F., Hoppe, B., Weber, L. T., Zschiedrich, S., Feldkamp, T., Oh, J., Bald, M., Schröppel, B., Holle, J. B., Jabs, W., Beckermann, J., Budde, K., Faulhaber-Walter, R., and Schaefer, F.

Published
2019
Full Text
View/download PDF

17. 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
Full Text
View/download PDF

18. 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
Full Text
View/download PDF

20. 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
Full Text
View/download PDF

25. [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

28. OP0102 IDENTIFICATION OF NEW RISK LOCI AND PATHWAYS INVOLVED IN GCA PATHOGENESIS BY A GENOME-WIDE STUDY

Author

Borrego-Yaniz, G., Ortiz-Fernández, L., Kerick, M., Madrid-Paredes, A., Vaglio, A., Hernández-Rodríguez, J., Mackie, S., Castañeda, S., Solans-Laqué, R., Mestre, J., Dasgupta, B., Watts, R., Khalidi, N., Langford, C., Ytterberg, S.R., Beretta, L., Govoni, M., Emmi, G., Cimmino, M.A., Witte, T., Neumann, T., Holle, J., Schönau, V., Pugnet, G., Papo, T., Haroche, J., Mahr, A., Mouthon, L., Molberg, Ø., Diamantopoulos, A., Voskuyl, A., Daikeler, T., Berger, C., Molloy, E., Blockmans, D., Consortium, U.G., Gca Consortium, I., Ortego, N., Brouwer, E., Lamprecht, P., Klapa, S., Salvarani, C., Merkel, P.A., Cid, M.C., González-Gay, M.A., Morgan, A., Martin Ibanez, J., and Márquez, A.

Published
2023
Full Text
View/download PDF

35. S2k Leitlinie Management der Großgefäßvaskulitiden

Author

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

Published
2020

38. Pediatr Nephrol

Author

HOLLE, J., QUERFELD, U., KIRCHNER, M., ANNINOS, A., OKUN, J., THURN-VALSASSINA, D., BAYAZIT, A., NIEMIRSKA, A., CANPOLAT, N., BULUT, I. K., DUZOVA, A., ANARAT, A., SHROFF, R., BILGINER, Y., CALISKAN, S., CANDAN, C., HARAMBAT, Jerome, OZCAKAR, Z. B., SOYLEMEZOGLU, O., TSCHUMI, S., HABBIG, S., YILMAZ, E., BALAT, A., ZUROWSKA, A., CAKAR, N., KRANZ, B., ERTAN, P., MELK, A., AZUKAITIS, K., and SCHAEFER, F.

Published
2019

42. Implantierbarer Atemschrittmacher — Funktionelle Elektrische Stimulation des Zwerchfells

Author

Mayr, W., Gerner, H., Girsch, W., Holle, J., Kluger, P., Meister, B., Moritz, E., Schwanda, G., Stöhr, H., Thoma, H., Hoffmann, G., Holzmann, D., Jäger, F., Riedling, K., Schwuttke, G. H., Pandelisev, K. A., White, R. C., Holzmann, H., Kausel, W., Nanz, G., Selberherr, S., Pötzl, H., Leopold, H., Röhrer, R., Winkler, G., Thurner, M., Seiner, K., Tritremmel, W., Walther, G., Schoitsch, Erwin, Hertl, S., Schaffar, G., Schmidt, K., Steinbrecher, H., Voggenberger, F., Windischhofer, W., Turba, R., Grabner, J., Aberl, H., Seifert, F., Buschbeck, F., Wallisch, K., Eichtinger, Ch., Wach, P., Bittinger, W., Kainz, A., Jestl, M., Beinstingl, W., Berthold, K., Köck, A., Gornik, E., Kloiber, G., Kreid, F., Schröcker, K. P., Schrödl, M., Brazda, E., Niedrist, G., Dietz, K., Fey, P., Frenkenberger, S., Furtner, M., Dejneka, B., Goiser, A., Sust, M., Kowatsch, M., Jorde, Ch., Thallinger, G. G., Mothwurf, E., Schubert, E., Steger, J., Trzeba, E., Awramow, I., Pucher, R., Auer, L. M., Schuy, S., Schima, H., Huber, L., Prodinger, A., Schmallegger, H., Thoma, H., Stöhr, H., Mayr, W., Steiner, R., Wiedenbauer, O., List, G., Wießpeiner, G., Petsch, Th., Doblhoff, G., Kirchner, D., Koudelka, O., Gierlinger, A., Ullrich, A., Braune, B., Horvat, H., Mayer, U., Gauby, A., Richter, A., Spiess, K. R., Klösch, W., Böck, N., Rosenkranz, H., Mitter, H., Hengl, M., Nussbaum, G., Gutternigh, K., Hahn, H., Koutny, P., Pühringer, K., Schwabach, H., Hirschler, M., Haas, M., Renner, A., Bratengeyer, E., Scholz-Nauendorff, C. C., Lebutsch, K., Volkmann, W., Gerner, H., Girsch, W., Holle, J., Kluger, P., Meister, B., Moritz, E., Schwanda, G., Prager, L., Wieβpeiner, G., Grübler, R., Dorfer, E., Oitzl, E., Pribyl, W., Bähring, M., Harter, J., and Sommer, D.

Published
1987
Full Text
View/download PDF

47. 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

48. Characteristics of Patients with atypical haemolytic-uremic Syndrome (aHUS) in Germany Data from the Global aHUS Registry

Author

Haas, C. S., Nitschke, M., Menne, J., Guthoff, M., Gaeckler, A., Bruck, H., Pape, L., Vester, U., Wuehl, E., Billing, H., Herbst, R., Thaiss, F., Hoppe, B., Weber, L. T., Zschiedrich, S., Feldkamp, T., Oh, J., Bald, M., Schroeppel, B., Holle, J. B., Jabs, W., Beckermann, J., Budde, K., Faulhaber-Walter, R., Schaefer, F., Haas, C. S., Nitschke, M., Menne, J., Guthoff, M., Gaeckler, A., Bruck, H., Pape, L., Vester, U., Wuehl, E., Billing, H., Herbst, R., Thaiss, F., Hoppe, B., Weber, L. T., Zschiedrich, S., Feldkamp, T., Oh, J., Bald, M., Schroeppel, B., Holle, J. B., Jabs, W., Beckermann, J., Budde, K., Faulhaber-Walter, R., and Schaefer, F.

Published
2019

50. Sicherheit und Wirksamkeitshinweise zum Off-label-Einsatz von Biologikatherapien nach Versagen konventioneller Therapien bei Patienten mit entzündlich rheumatischen Erkrankungen

Author

Proft, F., Schulze-Koops, H., Grunke, M., Schrezenmeier, E., Halleck, F., Henes, J., Unger, L., Schmidt, E., Fiehn, C., Jacobi, A., Iking-Konert, C., Kneitz, C., Schmidt, R.E., Bannert, B., Voll, R.E., Fischer-Betz, R., Kötter, I., Tony, H.P., Holle, J., Aringer, M., Erler, A., Behrens, F., Burmester, G.R., Dörner, T., and Publica

Abstract

Hintergrund GRAID2 (German Registry of Autoimmune Diseases 2) ist eine retrospektive, nichtinterventionelle, multizentrische Beobachtungsstudie, welche Sicherheits- und Wirksamkeitshinweise zum Einsatz von Biologikatherapien außerhalb der Zulassungsindikationen bei Patienten mit Autoimmunerkrankungen sowie bei Nierentransplantation zur Therapie bzw. der Prophylaxe einer Transplantatabstoßung, nach Versagen konventioneller Therapien erfasste. Ergebnisse Insgesamt wurden 311 Patienten dokumentiert. Die am häufigsten dokumentierten Erkrankungen waren neben der Sondersituation Nierentransplantations(NTx)-Rejektionsprophylaxe (18,3 %) Patienten mit ANCA-assoziierten Vaskulitiden (17,4 %), systemischem Lupus erythematodes (10,3 %), rekurrierenden Fiebersyndromen (8,4 %), Poly /Dermatomyositis (7,4 %), Pemphigus vulgaris (5,8 %), weiteren Arthritiden (Non-RA/ankylosierende Spondylitis/Psoriasisarthritis) sowie Großgefäßvaskulitiden und Polymyalgia rheumatica (4,5 %). Die Biologikatherapien umfassten in absteigender Häufigkeit Rituximab (RTX) (70,1 %), Tocilizumab (TCZ) (9,3 %), Infliximab (IFX) (7,1 %), Anakinra (ANK) (5,5 %), Adalimumab (ADA) (3,5 %), Etanercept (ETA) (2,3 %) und Certolizumab (CTZ) (0,6 %). Der dokumentierte Gesamtbeobachtungszeitraum unter Biologikatherapie betrug 338,5 Patientenjahre, bei einem mittleren Alter der Patienten von 47,8 Jahren und einem Anteil weiblicher Patientinnen von 56,9 %. Nach der ersten Gabe der Medikamente wurde deren Verträglichkeit vom behandelnden Arzt bei 95,5 % der Patienten als ""gut"" oder ""sehr gut"" eingeschätzt. Im Beobachtungsverlauf traten 275 unerwünschte Ereignisse auf mit 104 als schwerwiegend klassifizierten Nebenwirkungen bei 62 Patienten. Bei 19 dieser 62 Patienten (30,6 %) wurden schwerwiegende Infektionen bzw. opportunistische Erkrankungen berichtet. Dies entspricht einer Gesamtrate von 5,6 schwerwiegenden Infektionen auf 100 Patientenjahre. Es traten 6 Todesfälle im Beobachtungsverlauf ein, wobei nur in 1 Fall ein Kausalzusammenhang mit der Behandlung als wahrscheinlich eingeschätzt wurde. Die Registerdaten unterstützen die klinische Bedeutung von Rituximab für schwere Fälle der Kleingefäßvaskulitiden sowie von Tocilizumab für die Großgefäßvaskulitiden, wobei diese Behandlungen vor der Zulassung dieser Substanzen insbesondere bei Patienten mit schweren, bzw. therapierefraktären Verläufen angewendet wurden. Schlussfolgerung Die erhobenen Daten zeigen keine Hinweise für neue Sicherheitsaspekte aus den bekannten Zulassungsindikationen der dokumentierten Biologikatherapien bei einem Einsatz außerhalb der zugelassenen Indikationen bei Patienten mit entzündlich rheumatischen Systemerkrankungen, die sich auf konventionelle Therapien refraktär gezeigt hatten.

Published
2018

Catalog

Books, media, physical & digital resources
See catalog results