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5. LBA0001 EFFICACY AND SAFETY OF UPADACITINIB IN PATIENTS WITH GIANT CELL ARTERITIS (SELECT-GCA): A DOUBLE-BLIND, RANDOMIZED CONTROLLED PHASE 3 TRIAL

Author

Blockmans, D., primary, Penn, S. K., additional, Setty, A., additional, Schmidt, W., additional, Rubbert-Roth, A., additional, Hauge, E. M., additional, Keen, H., additional, Ishii, T., additional, Khalidi, N., additional, Liu, M., additional, Zhao, W., additional, Lagunes, I., additional, Romero, A., additional, Wung, P. K., additional, and Merkel, P. A., additional

Published
2024
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6. Flemish network on rare connective tissue diseases (CTD):patient pathways in systemic sclerosis. First steps taken

Author

Piette, Y., Van den Bossche, F., Aerts, J., Aerts, N., Ajeganova, S., Badot, V., Berghen, N., Blockmans, D., Brusselle, G., Caeyers, N., De Decker, M., De Haes, P., De Cock, C., De Keyser, F., De Langhe, E., Delcroix, M., De Nutte, H., De Pauw, M., Depicker, A., De Sutter, A., De Sutter, J., Du Four, T., Frank, C., Goubau, J., Guiot, J., Gutermuth, J., Heeman, L., Houssiau, F., Hennes, I., Lenaerts, J., Lintermans, A., Loeys, B., Luyten, H., Maeyaert, B., Malfait, F., Moeyersoons, A., Mostmans, Y., Nijs, J., Poppe, B., Polfliet, K., Ruttens, D., Sabato, V., Schoeters, E., Slabbynck, H., Stuer, A., Tamirou, F., Thevissen, Kristof, Van Kersschaever, G., Vanneuville, B., Van Offel, J., Vanthuyne, M., Van Wabeke, J., Verbist, C., Vos, I., Westhovens, R., Wuyts, W., Yserbyt, J., Smith, V., Piette, Y., Van den Bossche, F., Aerts, J., Aerts, N., Ajeganova, S., Badot, V., Berghen, N., Blockmans, D., Brusselle, G., Caeyers, N., De Decker, M., De Haes, P., De Cock, C., De Keyser, F., De Langhe, E., Delcroix, M., De Nutte, H., De Pauw, M., Depicker, A., De Sutter, A., De Sutter, J., Du Four, T., Frank, C., Goubau, J., Guiot, J., Gutermuth, J., Heeman, L., Houssiau, F., Hennes, I., Lenaerts, J., Lintermans, A., Loeys, B., Luyten, H., Maeyaert, B., Malfait, F., Moeyersoons, A., Mostmans, Y., Nijs, J., Poppe, B., Polfliet, K., Ruttens, D., Sabato, V., Schoeters, E., Slabbynck, H., Stuer, A., Tamirou, F., Thevissen, Kristof, Van Kersschaever, G., Vanneuville, B., Van Offel, J., Vanthuyne, M., Van Wabeke, J., Verbist, C., Vos, I., Westhovens, R., Wuyts, W., Yserbyt, J., and Smith, V.

Abstract

Despite the low prevalence of each rare disease, the total burden is high. Patients with rare diseases encounter numerous barriers, including delayed diagnosis and limited access to high-quality treatments. In order to tackle these challenges, the European Commission launched the European Reference Networks (ERNs), cross-border networks of healthcare providers and patients representatives. In parallel, the aims and structure of these ERNs were translated at the federal and regional levels, resulting in the creation of the Flemish Network of Rare Diseases. In line with the mission of the ERNs and to ensure equal access to care, we describe as first patient pathways for systemic sclerosis (SSc), as a pilot model for other rare connective and musculoskeletal diseases. Consensus was reached on following key messages: 1. Patients with SSc should have multidisciplinary clinical and investigational evaluations in a tertiary reference expert centre at baseline, and subsequently every three to 5 years. Intermediately, a yearly clinical evaluation should be provided in the reference centre, whilst SSc technical evaluations are permissionably executed in a centre that follows SSc-specific clinical practice guidelines. In between, monitoring can take place in secondary care units, under the condition that qualitative examinations and care including interactive multidisciplinary consultations can be provided. 2. Patients with early diffuse cutaneous SSc, (progressive) interstitial lung disease and/or pulmonary arterial hypertension should undergo regular evaluations in specialised tertiary care reference institutions. 3. Monitoring of patients with progressive interstitial lung disease and/or pulmonary (arterial) hypertension will be done in agreement with experts of ERN LUNG.

Published
2024

7. Diagnostic Approaches for Large Vessel Vasculitides

Author

Betrains A and Blockmans D

Subjects
large vessel vasculitis – giant cell arteritis – takayasu arteritis – periaortitis – pet-scan – ultrasonography – temporal artery biopsy, Diseases of the musculoskeletal system, RC925-935
Abstract

Albrecht Betrains,1,2 Daniel Blockmans1,2 1Department of General Internal Medicine, University Hospitals Leuven, Leuven, Belgium; 2Department of Microbiology, Immunology and Transplantation, Laboratory of Clinical Infectious and Inflammatory Disorders, KU Leuven, Leuven, BelgiumCorrespondence: Daniel BlockmansDepartment of General Internal Medicine, University Hospitals Leuven, Herestraat 49, Leuven, 3000, BelgiumTel +3216344279Email daniel.blockmans@uzleuven.beAbstract: The large vessel vasculitides comprise giant cell arteritis (GCA), Takayasu arteritis (TAK), and chronic periaortitis. The diagnostic approach to these conditions involves the correct use and interpretation of clinical criteria, imaging techniques, and, in case of GCA, temporal artery biopsy. Ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT) reveal a homogeneous, concentric, thickening of the arterial wall. MRI and CT may also reveal aneurysms and stenoses. 18F-Fluorodeoxyglucose (FDG)-PET shows increased FDG uptake of inflamed artery walls delineating increased metabolic activity. Ultrasound, FDG-PET, and MRI are the recommended imaging techniques in GCA and TAK. In patients with a high suspicion of GCA who present with visual disturbances, initiation of high-dose intravenous corticosteroids should not be delayed by imaging. Extracranial large vessel vasculitis may be confirmed by all three modalities, particularly by FDG-PET in case of atypical clinical pictures. In this article, we review the role of the GCA and TAK ACR classification criteria, temporal artery biopsy, conventional angiography, ultrasound, MRI, magnetic resonance angiography (MRA), CT angiography (CTA), and FDG-PET in the diagnostic approach of large vessel vasculitis.Keywords: large vessel vasculitis, giant cell arteritis, Takayasu arteritis, periaortitis, PET-scan, ultrasonography, temporal artery biopsy

Published
2021

8. De thorax

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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9. Methoden van het lichamelijk onderzoek

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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10. Het algemeen onderzoek

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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11. Hoofd en hals

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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12. De algemene anamnese

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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13. De anamnese gericht op de hoofdklacht

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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14. Veelvoorkomende klachten

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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15. Het diagnostisch proces

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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16. De anamnese: algemene principes

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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17. Het patiëntendossier

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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18. De geriatrische patiënt

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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19. Het neurologisch onderzoek

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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20. De gewrichten

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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21. De extremiteiten

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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22. Genitaliën en rectum

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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23. De buik

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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24. Flemish network on rare connective tissue diseases (CTD): patient pathways in systemic sclerosis. First steps taken

Author

Piette, Y., primary, Van den Bossche, F., additional, Aerts, J., additional, Aerts, N., additional, Ajeganova, S., additional, Badot, V., additional, Berghen, N., additional, Blockmans, D., additional, Brusselle, G., additional, Caeyers, N., additional, De Decker, M., additional, De Haes, P., additional, De Cock, C., additional, De Keyser, F., additional, De Langhe, E., additional, Delcroix, M., additional, De Nutte, H., additional, De Pauw, M., additional, Depicker, A., additional, De Sutter, A., additional, De Sutter, J., additional, Du Four, T., additional, Frank, C., additional, Goubau, J., additional, Guiot, J., additional, Gutermuth, J., additional, Heeman, L., additional, Houssiau, F., additional, Hennes, I., additional, Lenaerts, J., additional, Lintermans, A., additional, Loeys, B., additional, Luyten, H., additional, Maeyaert, B., additional, Malfait, F., additional, Moeyersoons, A., additional, Mostmans, Y., additional, Nijs, J., additional, Poppe, B., additional, Polfliet, K., additional, Ruttens, D., additional, Sabato, V., additional, Schoeters, E., additional, Slabbynck, H., additional, Stuer, A., additional, Tamirou, F., additional, Thevissen, Kristof, additional, Van Kersschaever, G., additional, Vanneuville, B., additional, Van Offel, J., additional, Vanthuyne, M., additional, Van Wabeke, J., additional, Verbist, C., additional, Vos, I., additional, Westhovens, R., additional, Wuyts, W., additional, Yserbyt, J., additional, and Smith, V., additional

Published
2023
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25. Cardiovasculaire aandoeningen

Author

Blockmans, D., Dens, J., Desmet, W. J. R., Ferdinande, P., Heidbüchel, H., Stockman, W., Vlasselaers, D., Weekers, F., van de Werf, F., Bruining, H.A., editor, Lauwers, P., editor, and Thijs, L.G., editor

Published
2017
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26. De patiënt-artsrelatie

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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27. De samenvatting van het onderzoek

Author

van der Meer, J.W.M, van der Meer, J., Linthorst, G., Postma, C.T., Blockmans, D., van der Meer, J.W.M, editor, van der Meer, J., editor, Linthorst, G., editor, Postma, C.T., editor, and Blockmans, D., editor

Published
2016
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32. 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
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33. LB0009 EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update

Author

Dejaco, C., primary, Ramiro, S., additional, Bond, M., additional, Bosch, P., additional, Ponte, C., additional, Mackie, S., additional, Bley, T., additional, Blockmans, D., additional, Brolin, S., additional, Bolek, E. C., additional, Cassie, R., additional, Cid, M. C., additional, Molina Collada, J., additional, Dasgupta, B., additional, Dalsgaard Nielsen, B., additional, De Miguel, E., additional, Direskeneli, H., additional, Duftner, C., additional, Hocevar, A., additional, Moltó, A., additional, Schäfer, V., additional, Seitz, L., additional, Slart, R., additional, and Schmidt, W., additional

Published
2023
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35. Efficacité et tolérance d'upadacitinib chez des patients atteints d'artérite à cellules géantes (SELECT-GCA) : essai de phase III contrôlé et randomisé en double aveugle.

Author

Blockmans, D., Penn, S., Setty, A., Schmidt, W., Rubbert-Roth, A., Hauge, E., Keen, H., Ishii, T., Khalidi, N., Liu, M., Zhao, W., Lagunes-Galindo, I., Romero, A.B., Wung, P.K., Devauchelle-Pensec, V., and Merkel, P.A.

Abstract

Les corticoïdes (GC) restent la principale option thérapeutique de l'artérite à cellules géantes (ACG). L'upadacitinib (UPA), un inhibiteur oral et sélectif de JAK, approuvé pour traiter plusieurs maladies inflammatoires, a le potentiel de traiter l'ACG. SELECT-GCA est une étude randomisée de phase III, en double aveugle contrôlée vs PBO dont l'objectif est d'évaluer l'efficacité et la tolérance d'UPA en association avec une diminution progressive des GC, chez des patients ayant une ACG. Sont rapportés ici les résultats de l'analyse principale à 52 semaines (S). Les patients ont reçu l'UPA 7,5 mg (UPA7,5) ou 15 mg (UPA15), avec une diminution des GC sur 26S ou un PBO avec une diminution des GC sur 52S. Les patients éligibles avaient ≥ 50 ans, un diagnostic ou une rechute d'ACG, avaient été traités par ≥ 40 mg/j de prednisone ou équivalent avant l'inclusion (incl), et étaient traités par prednisone ≥ 20 mg/j à l'incl. Les patients traités antérieurement par inhibiteurs de JAK, ayant eu une réponse inadéquate ou traités par des anti-IL-6 au cours des 4S précédant l'incl ou ayant une utilisation chronique de GC systémiques étaient exclus. Le critère principal était la rémission maintenue, définie par l'absence de signes ou symptômes d'ACG entre S12 et S52 et le respect du schéma de réduction des GC prévu au protocole. Les critères secondaires incluaient la rémission complète maintenue (rémission maintenue avec normalisation de la VS et de la CRP), des critères liés aux poussées de la maladie, et l'exposition cumulée aux GC. Les événements indésirables (EI) ont été documentés durant les 52S. Au total, 428 patients ont été randomisés et traités (PBO : n = 112 ; UPA7,5 : n = 107 ; UPA15 : n = 209). Les caractéristiques à l'incl étaient comparables entre les groupes de traitement. Le critère principal de rémission maintenue à S52 a été atteint avec l'UPA15 vs le PBO (46 % vs 29 %, p = 0,0019) et 9/11 critères secondaires contrôlés pour la multiplicité des tests ont été atteints avec l'UPA15, y compris la rémission complète maintenue de S12 à S52 (37 % vs 16 %, p < 0,0001). Une diminution du risque de poussées a été observée avec l'UPA15 par rapport au PBO au cours des 52S (Fig. 1). L'exposition médiane cumulée aux GC sur les 52S a été significativement plus faible avec l'UPA15 vs le PBO (1615 mg vs 2882 mg, p < 0,0001). Pour la plupart des critères, une amélioration numériquement supérieure à celle du PBO a été observée avec l'UPA7,5. Les résultats de tolérance étaient généralement similaires dans les groupes UPA et PBO (Tableau 1), avec des taux plus élevés d'infections graves et de MACE rapportés dans le groupe PBO et aucun MACE rapporté dans les groupes UPA. Les taux de zona, lymphopénie, anémie et cancer cutané non-mélanome (CCNM) étaient numériquement plus élevés avec UPA15 vs PBO. Les taux d'évènements thromboemboliques étaient comparables dans tous les groupes de traitement. Les taux d'EI graves et de cancers (hors CCNM) étaient similaires entre UPA15 et le PBO. Quatre décès ont été rapportés : deux dans le groupe PBO et deux dans le groupe UPA15 (un attribuable au COVID-19 et un de cause inexpliquée). L'UPA15 a démontré une efficacité supérieure et une utilisation réduite des GC par rapport au PBO. Aucun nouveau signal de tolérance n'a été identifié avec UPA par rapport au profil connu [1]. L'UPA15 a présenté un rapport bénéfice/risque favorable et représente un nouveau traitement ciblé potentiel pour les patients atteints d'ACG. [ABSTRACT FROM AUTHOR]

Published
2024
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37. 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
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38. Efficacy and safety of mavrilimumab in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial

Author

Cid, MC, Unizony, SH, Blockmans, D, Brouwer, E, Dagna, L, Dasgupta, B, Hellmich, B, Molloy, E, Salvarani, C, Trapnell, BC, Warrington, KJ, Wicks, I, Samant, M, Zhou, T, Pupim, L, Paolini, JF, Cid, MC, Unizony, SH, Blockmans, D, Brouwer, E, Dagna, L, Dasgupta, B, Hellmich, B, Molloy, E, Salvarani, C, Trapnell, BC, Warrington, KJ, Wicks, I, Samant, M, Zhou, T, Pupim, L, and Paolini, JF

Abstract

OBJECTIVES: Granulocyte-macrophage colony-stimulating factor (GM-CSF) is implicated in pathogenesis of giant cell arteritis. We evaluated the efficacy of the GM-CSF receptor antagonist mavrilimumab in maintaining disease remission. METHODS: This phase 2, double-blind, placebo-controlled trial enrolled patients with biopsy-confirmed or imaging-confirmed giant cell arteritis in 50 centres (North America, Europe, Australia). Active disease within 6 weeks of baseline was required for inclusion. Patients in glucocorticoid-induced remission were randomly assigned (3:2 ratio) to mavrilimumab 150 mg or placebo injected subcutaneously every 2 weeks. Both groups received a 26-week prednisone taper. The primary outcome was time to adjudicated flare by week 26. A prespecified secondary efficacy outcome was sustained remission at week 26 by Kaplan-Meier estimation. Safety was also assessed. RESULTS: Of 42 mavrilimumab recipients, flare occurred in 19% (n=8). Of 28 placebo recipients, flare occurred in 46% (n=13). Median time to flare (primary outcome) was 25.1 weeks in the placebo group, but the median was not reached in the mavrilimumab group (HR 0.38; 95% CI 0.15 to 0.92; p=0.026). Sustained remission at week 26 was 83% for mavrilimumab and 50% for placebo recipients (p=0.0038). Adverse events occurred in 78.6% (n=33) of mavrilimumab and 89.3% (n=25) of placebo recipients. No deaths or vision loss occurred in either group. CONCLUSIONS: Mavrilimumab plus 26 weeks of prednisone was superior to placebo plus 26 weeks of prednisone for time to flare by week 26 and sustained remission in patients with giant cell arteritis. Longer treatment is needed to determine response durability and quantify the glucocorticoid-sparing potential of mavrilimumab. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov number: NCT03827018, Europe (EUdraCT number: 2018-001003-36), and Australia (CT-2018-CTN-01 865-1).

Published
2022

40. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis

Author

Grayson, P. C., Ponte, C., Suppiah, R., Robson, J. C., Craven, A., Judge, A., Khalid, S., Hutchings, A., Luqmani, R. A., Watts, R. A., Merkel, P. A., Gatenby, P., Hill, C., Ranganathan, D., Kronbichler, A., Blockmans, D., Barra, L., Carette, S., Pagnoux, C., Dhindsa, N., Fifi-Mah, A., Khalidi, N., Liang, P., Milman, N., Pineau, C., Tian, X., Wang, G., Wang, T., Zhao, M. -H., Tesar, V., Baslund, B., Hammam, N., Shahin, A., Pirila, L., Putaala, J., Hellmich, B., Henes, J., Lamprecht, P., Neumann, T., Schmidt, W., Sunderkoetter, C., Szekanecz, Z., Danda, D., Das, S., Gupta, R., Rajasekhar, L., Sharma, A., Wagh, S., Clarkson, M., Molloy, E., Salvarani, C., Schiavon, F., Tombetti, E., Vaglio, A., Amano, K., Arimura, Y., Dobashi, H., Fujimoto, S., Harigai, M., Hirano, F., Hirahashi, J., Honma, S., Kawakami, T., Kobayashi, S., Kono, H., Makino, H., Matsui, K., Muso, E., Suzuki, K., Ikeda, K., Takeuchi, T., Tsukamoto, T., Uchida, S., Wada, T., Yamada, H., Yamagata, K., Yumura, W., Lai, K. S., Flores-Suarez, L. F., Hinojosa, A., Rutgers, B., Tak, P. -P., Grainger, R., Quincey, V., Stamp, L., Besada, E., Diamantopoulos, A., Sznajd, J., Azevedo, E., Geraldes, R., Rodrigues, M., Santos, E., Song, Y. -W., Moiseev, S., Hocevar, A., Cid, M. C., Moreno, X. S., Atukorala, I., Berglin, E., Mohammed, A., Segelmark, M., Daikeler, T., Direskeneli, H., Hatemi, G., Kamali, S., Karadag, O., Pehlevan, S., Adler, M., Basu, N., Bruce, I., Chakravarty, K., Dasgupta, B., Flossmann, O., Gendi, N., Hassan, A., Hoyles, R., Jayne, D., Jones, C., Klocke, R., Lanyon, P., Laversuch, C., Luqmani, R., Robson, J., Magliano, M., Mason, J., Maw, W. W., Mcinnes, I., Mclaren, J., Morgan, M., Morgan, A., Mukhtyar, C., O'Riordan, E., Patel, S., Peall, A., Venkatachalam, S., Vermaak, E., Menon, A., Watts, R., Yee, C. -S., Albert, D., Calabrese, L., Chung, S., Forbess, L., Gaffo, A., Gewurz-Singer, O., Grayson, P., Liang, K., Matteson, E., Springer, J., Sreih, A., and Translational Immunology Groningen (TRIGR)

Subjects
Adult, Male, Vasculitis, Myeloblastin, Immunology, Churg-Strauss Syndrome, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Antibodies, Antineutrophil Cytoplasmic, Diagnosis, Differential, Rheumatology, Risk Factors, Humans, Immunology and Allergy, anti-neutrophil cytoplasm antibody, Prospective Studies, Aged, Granulomatosis with Polyangiitis, Reproducibility of Results, Middle Aged, United States, Female, eosinophilic granulomatosis with polyangiitis, Eosinophilic Granuloma, Europe, classification, Societies
Abstract

ObjectiveTo develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.ResultsThe development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×109/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3–ANCA positivity (−3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (−1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%).ConclusionThe 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.

Published
2022
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41. Subclinical giant cell arteritis in new onset polymyalgia rheumatica A systematic review and meta-analysis of individual patient data

Author

Hemmig, AK, Gozzoli, D, Werlen, L, Ewald, H, Aschwanden, M, Blockmans, D, Brouwer, E, Buchanan, RRC, Camellino, D, Campochiaro, C, Cimmino, MA, Corominas, H, Gloy, V, Henckaerts, L, Kyburz, D, Moya-Alvarado, P, Owen, CE, Stegert, M, Tomelleri, A, van Sleen, Y, Yamashita, H, Imfeld, S, Berger, CT, Hemkens, LG, and Daikeler, T

Subjects
Meta-analysis, Polymyalgia rheumatica, Subclinical vasculitis, Systematic review, Giant cell arteritis
Abstract

Objectives: To determine the prevalence and predictors of subclinical giant cell arteritis (GCA) in patients with newly diagnosed polymyalgia rheumatica (PMR). Methods: PubMed, Embase, and Web of Science Core Collection were systematically searched (date of last search July 14, 2021) for any published information on any consecutively recruited cohort reporting the prevalence of GCA in steroid-na & iuml;ve patients with PMR without cranial or ischemic symptoms. We combined prevalences across populations in a random-effect meta-analysis. Potential predictors of subclinical GCA were identified by mixed effect logistic regression using individual patient data (IPD) from cohorts screened with PET/(CT). Results: We included 13 cohorts with 566 patients from studies published between 1965 to 2020. Subclinical GCA was diagnosed by temporal artery biopsy in three studies, ultrasound in three studies, and PET/(CT) in seven studies. The pooled prevalence of subclinical GCA across all studies was 23% (95% CI 14%-36%, I-2=84%) for any screening method and 29% in the studies using PET/(CT) (95% CI 13%-53%, I-2=85%) (n=266 patients). For seven cohorts we obtained IPD for 243 patients screened with PET/(CT). Inflammatory back pain (OR 2.73, 1.32-5.64), absence of lower limb pain (OR 2.35, 1.05-5.26), female sex (OR 2.31, 1.17-4.58), temperature >37 degrees (OR 1.83, 0.90-3.71), weight loss (OR 1.83, 0.96-3.51), thrombocyte count (OR 1.51, 1.05-2.18), and haemoglobin level (OR 0.80, 0.64-1.00) were most strongly associated with subclinical GCA in the univariable analysis but not C-reactive protein (OR 1.00, 1.00-1.01) or erythrocyte sedimentation rate (OR 1.01, 1.00-1.02). A prediction model calculated from these variables had an area under the curve of 0.66 (95% CI 0.55-0.75). Conclusion: More than a quarter of patients with PMR may have subclinical GCA. The prediction model from the most extensive IPD set has only modest diagnostic accuracy. Hence, a paradigm shift in the assessment of PMR patients in favour of implementing imaging studies should be discussed.

Published
2022

44. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon K, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phillip A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Schleinitz, Nicolas, Takahashi, Naoki, Umehara, Hisanori, Zen, Yoh, Stone, Collaborators: Akamizu T, John H., Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chiba, T, Cornell, L, Culver, E, Darabian, S, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, Luca, Hernandez-Molina, G, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Leng, H, Lerch, Mm, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Nakamura, S, Nordeide, J, Notohara, K, Paira, S, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Sato, Y, Sekiguchi, H, Sokol, Ev, Stone, Jr, Sun, W, Takahashi, H, Takahira, M, Tanaka, Y, Vaglio, A, Villamil, A, Wada, Y, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, Hk, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Schleinitz, N, Takahashi, N, Umehara, H, Zen, Y, Stone, Jh, and Members of the ACR/EULAR IgG4-RD Classification Criteria Working, Group.

Subjects
rheumatoid arthritis, Adult, Male, 0301 basic medicine, medicine.medical_specialty, Immunology, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, parasitic diseases, Epidemiology, Humans, Immunology and Allergy, Medicine, Pathological, Sjøgren's syndrome, Aged, 030203 arthritis & rheumatology, business.industry, Middle Aged, inflammation, medicine.disease, Test (assessment), 030104 developmental biology, Rheumatoid arthritis, Radiological weapon, Female, Immunoglobulin G4-Related Disease, business, Rheumatism, Decision analysis
Abstract

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

Published
2019
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45. Harmonization of antineutrophil cytoplasmic antibodies (ANCA) testing by reporting test result-specific likelihood ratios: position paper

Author

Bossuyt, X., Bossuyt, X., Damoiseaux, J., Rasmussen, N., van Paassen, P., Hellmich, B., Baslund, B., Blockmans, D., Vermeersch, P., Lopez-Hoyos, M., Vercammen, M., Barret, E., Hammar, F., Leinfelder, U., Mahler, M., Olschowka, N., Roggenbuck, D., Schlumberger, W., Walker, R., Ronnelid, J., Tervaert, J.W.C., Csernok, E., Fierz, W., European Federation of Laboratory Medicine (EFLM) Task and Finish Group “Autoimmunity Testing”, European Autoimmune Standardization Initiative (EASI), European Consensus Finding Study Group on autoantibodies (ECFSG), Bossuyt, X., Bossuyt, X., Damoiseaux, J., Rasmussen, N., van Paassen, P., Hellmich, B., Baslund, B., Blockmans, D., Vermeersch, P., Lopez-Hoyos, M., Vercammen, M., Barret, E., Hammar, F., Leinfelder, U., Mahler, M., Olschowka, N., Roggenbuck, D., Schlumberger, W., Walker, R., Ronnelid, J., Tervaert, J.W.C., Csernok, E., Fierz, W., European Federation of Laboratory Medicine (EFLM) Task and Finish Group “Autoimmunity Testing”, European Autoimmune Standardization Initiative (EASI), and European Consensus Finding Study Group on autoantibodies (ECFSG)

Published
2021

46. Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases

Author

Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, Karras, A, Durel, Cécile-Audrey, Sinico, Renato A, Teixeira, Vitor, Jayne, David, Belenfant, Xavier, Marchand-Adam, Sylvain, Pugnet, Gregory, Gaultier, Jacques, Le Gallou, Thomas, Titeca-Beauport, Dimitri, Agard, Christian, Barbet, Christelle, Bardy, Antoine, Blockmans, Daniel, Boffa, Jean-Jacques, Bouet, Julien, Cottin, Vincent, Crabol, Yoann, Deligny, Christophe, Essig, Marie, Godmer, Pascal, Guilpain, Philippe, Hirschi-Santelmo, Sandrine, Rafat, Cédric, Puéchal, Xavier, Taillé, Camille, Karras, Alexandre, Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, Karras, A, Durel, Cécile-Audrey, Sinico, Renato A, Teixeira, Vitor, Jayne, David, Belenfant, Xavier, Marchand-Adam, Sylvain, Pugnet, Gregory, Gaultier, Jacques, Le Gallou, Thomas, Titeca-Beauport, Dimitri, Agard, Christian, Barbet, Christelle, Bardy, Antoine, Blockmans, Daniel, Boffa, Jean-Jacques, Bouet, Julien, Cottin, Vincent, Crabol, Yoann, Deligny, Christophe, Essig, Marie, Godmer, Pascal, Guilpain, Philippe, Hirschi-Santelmo, Sandrine, Rafat, Cédric, Puéchal, Xavier, Taillé, Camille, and Karras, Alexandre

Abstract

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods: We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results: Sixty-three patients [27 women, median age 60 years (18-83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1-296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion: Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.

Published
2021

48. POS0806 FINDINGS CONSISTENT WITH SUBCLINICAL VASCULITIS IN PATIENTS WITH NEW ONSET POLYMYALGIA: A SYSTEMATIC LITERATURE REVIEW AND A META-ANALYSIS OF COHORT DATA

Author

Gozzoli, D. S., primary, Hemmig, A., additional, Hemkens, L., additional, Werlen, L., additional, Ewald, H., additional, Berger, C., additional, Kyburz, D., additional, Imfeld, S., additional, Aschwanden, M., additional, Stegert, M., additional, Camellino, D., additional, Cimmino, M. A., additional, Campochiaro, C., additional, Tomelleri, A., additional, Henckaerts, L., additional, Blockmans, D., additional, Moya, P., additional, Corominas, H., additional, Buchanan, R., additional, Owen, C., additional, Van Sleen, Y., additional, Brouwer, E., additional, Ymashita, H., additional, and Daikeler, T., additional

Published
2021
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