Evidence-based guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis

Emmi, Giacomo, Bettiol, Alessandra, Gelain, Elena, Bajema, Ingeborg M., Berti, Alvise, Burns, Stella, Cid, Maria C., Cohen Tervaert, Jan W., Cottin, Vincent, Durante, Eugenia, Holle, Julia U., Mahr, Alfred D., Del Pero, Marcos Martinez, Marvisi, Chiara, Mills, John, Moiseev, Sergey, Moosig, Frank, Mukhtyar, Chetan ORCID: https://orcid.org/0000-0002-9771-6667, Neumann, Thomas, Olivotto, Iacopo, Salvarani, Carlo, Seeliger, Benjamin, Sinico, Renato A., Taillé, Camille, Terrier, Benjamin, Venhoff, Nils, Bertsias, George, Guillevin, Loïc, Jayne, David R.W. and Vaglio, Augusto (2023) Evidence-based guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nature Reviews Rheumatology, 19 (6). pp. 378-393. ISSN 1759-4790

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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.

Item Type: Article
Additional Information: Funding Information: The authors thank M. Tesi for preparing Fig. 1 , and F. Bello, A. Biscarini and M. Zampieri for helping to source CT and MR images. The authors also thank the European EGPA study group members who participated in the 4th EESG meeting at the 20th International Vasculitis and ANCA Workshop (Dublin, April 2022) and contributed to the discussion of the current guideline. Publisher Copyright: © 2023, Springer Nature Limited.
Uncontrolled Keywords: rheumatology ,/dk/atira/pure/subjectarea/asjc/2700/2745
Faculty \ School: Faculty of Medicine and Health Sciences > Norwich Medical School
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Depositing User: LivePure Connector
Date Deposited: 22 Oct 2023 01:09
Last Modified: 10 Nov 2023 01:38
URI: https://ueaeprints.uea.ac.uk/id/eprint/93394
DOI: 10.1038/s41584-023-00958-w

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