Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.

Publication

Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.

Journal Paper/Review - May 9, 2023

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

Citation

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. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 2023; 19:378-393.

Type

Journal Paper/Review (English)

Journal

Nat Rev Rheumatol 2023; 19

Publication Date

May 9, 2023

Issn Electronic

1759-4804

Pages

378-393

Brief description/objective

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.