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

Giacomo Emmi, Alessandra Bettiol, Elena Gelain, Ingeborg M. Bajema, Alvise Berti, Stella Burns, Maria C. Cid, Jan W. Cohen Tervaert, Vincent Cottin, Eugenia Durante, Julia U. Holle, Alfred D. Mahr, Marcos Martinez Del Pero, Chiara Marvisi, John Mills, Sergey Moiseev, Frank Moosig, Chetan Mukhtyar, Thomas Neumann, Iacopo OlivottoCarlo Salvarani, Benjamin Seeliger, Renato A. Sinico, Camille Taillé, Benjamin Terrier, Nils Venhoff, George Bertsias, Loïc Guillevin, David R.W. Jayne, Augusto Vaglio

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8 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)378-393
Number of pages16
JournalNature Reviews Rheumatology
Issue number6
Early online date9 May 2023
Publication statusPublished - Jun 2023

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