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 Olivotto, Carlo Salvarani, Benjamin Seeliger, Renato A Sinico, Camille Taillé, Benjamin Terrier, Nils Venhoff, George Bertsias, Loïc Guillevin, David R W Jayne, Augusto Vaglio
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...
May 9, 2023: Nature Reviews. Rheumatology