TY - JOUR
T1 - Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis
AU - Emmi, Giacomo
AU - Bettiol, Alessandra
AU - Gelain, Elena
AU - Bajema, Ingeborg M.
AU - Berti, Alvise
AU - Burns, Stella
AU - Cid, Maria C.
AU - Cohen Tervaert, Jan W.
AU - Cottin, Vincent
AU - Durante, Eugenia
AU - Holle, Julia U.
AU - Mahr, Alfred D.
AU - Del Pero, Marcos Martinez
AU - Marvisi, Chiara
AU - Mills, John
AU - Moiseev, Sergey
AU - Moosig, Frank
AU - Mukhtyar, Chetan
AU - Neumann, Thomas
AU - Olivotto, Iacopo
AU - Salvarani, Carlo
AU - Terrier, Benjamin
AU - Sinico, Renato A.
AU - Taille, Camille
AU - Venhoff, Nils
AU - Bertsias, George
AU - Guillevin, Loic
AU - Jayne, David R. W.
AU - Vaglio, Augusto
PY - 2023/6/1
Y1 - 2023/6/1
N2 - This article presents the first Evidence-Based Guideline dedicated specifically to the diagnosis and management of eosinophilic granulomatosis with polyangiitis. The 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of eosinophilic granulomatosis with polyangiitis.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.
AB - This article presents the first Evidence-Based Guideline dedicated specifically to the diagnosis and management of eosinophilic granulomatosis with polyangiitis. The 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of eosinophilic granulomatosis with polyangiitis.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.
KW - CHURG-STRAUSS-SYNDROME
KW - ANCA-ASSOCIATED VASCULITIS
KW - ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES
KW - SYSTEMIC-NECROTIZING-VASCULITIDES
KW - VENOUS THROMBOEMBOLIC EVENTS
KW - TERM-FOLLOW-UP
KW - POLYARTERITIS-NODOSA
KW - MICROSCOPIC POLYANGIITIS
KW - WEGENERS-GRANULOMATOSIS
KW - POOR-PROGNOSIS
U2 - 10.1038/s41584-023-00958-w
DO - 10.1038/s41584-023-00958-w
M3 - Article
C2 - 37161084
SN - 1759-4790
VL - 19
SP - 378
EP - 393
JO - Nature Reviews Rheumatology
JF - Nature Reviews Rheumatology
IS - 6
ER -