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

被引：138

作者：

Emmi, Giacomo ^{[1
,2
]}

Bettiol, Alessandra ^{[1
]}

Gelain, Elena ^{[3
]}

Bajema, Ingeborg M. ^{[4
]}

Berti, Alvise ^{[5
,6
]}

Burns, Stella ^{[7
]}

Cid, Maria C. ^{[8
,9
]}

Cohen Tervaert, Jan W. ^{[10
,11
]}

Cottin, Vincent ^{[12
]}

Durante, Eugenia ^{[13
]}

Holle, Julia U. ^{[14
]}

Mahr, Alfred D. ^{[15
]}

Del Pero, Marcos Martinez ^{[7
,16
]}

Marvisi, Chiara ^{[17
]}

Mills, John ^{[18
]}

Moiseev, Sergey ^{[19
]}

Moosig, Frank ^{[14
]}

Mukhtyar, Chetan ^{[20
]}

Neumann, Thomas ^{[15
]}

Olivotto, Iacopo ^{[21
,22
]}

Salvarani, Carlo ^{[23
,24
]}

Terrier, Benjamin ^{[25
,26
,30
]}

Sinico, Renato A. ^{[27
,28
]}

Taille, Camille ^{[29
]}

Terrier, Benjamin ^{[25
,26
,30
]}

Venhoff, Nils ^{[31
]}

Bertsias, George ^{[32
,33
]}

Guillevin, Loic ^{[34
]}

Jayne, David R. W. ^{[35
,36
]}

Vaglio, Augusto ^{[3
,37
]}

机构：

[1] Univ Florence, Dept Expt & Clin Med, Florence, Italy

[2] Monash Univ, Ctr Inflammatory Dis, Monash Med Ctr, Dept Med, Clayton, Vic, Australia

[3] Meyer Childrens Hosp IRCCS, Nephrol & Dialysis Unit, Florence, Italy

[4] Univ Groningen, Dept Pathol, Med Ctr, Groningen, Netherlands

[5] Santa Chiara Reg Hosp, Rheumatol, APSS Trento, Trento, Italy

[6] Univ Trento, Dept Cellular Computat & Integrat Biol CIBIO, Trento, Italy

[7] Cambridge Univ Hosp NHS Fdn Trust, Vasculitis & Lupus Clin, Cambridge, England

[8] Hosp Clin Barcelona, Dept Autoimmune Dis, Barcelona, Spain

[9] Univ Barcelona, Inst Invest Biomed August Pi & Sunyer, Barcelona, Spain

[10] Univ Alberta, Dept Med, Div Rheumatol, Edmonton, AB, Canada

[11] Maastricht Univ, Sch Mental Hlth & Neurosci, Maastricht, Netherlands

[12] Univ Lyon, Claude Bernard Univ Lyon 1, Natl Reference Ctr Rare Pulm Dis, Louis Pradel Hosp,Hosp Civils Lyon,IVPC,INRAE,ERN, Lyon, France

[13] Assoc Pazienti Con Sindrome Churg Strauss, APACS, Arosio, Italy

[14] Rheumazentrum Schleswig Holstein Mitte, Neumunster Kiel, Germany

[15] Cantonal Hosp St Gallen, Dept Rheumatol, St Gallen, Switzerland

[16] West Suffolk Hosp, ENT Dept, Bury St Edmunds, Suffolk, England

[17] Univ Modena & Reggio Emilia, Rheumatol Unit, Modena, Italy

[18] Vasculitis UK, Winster, England

[19] Sechenov First Moscow State Med Univ, Tareev Clin Internal Dis, Moscow, Russia

[20] Norfolk & Norwich Univ Hosp NHS Trust, Rheumatol Dept, Vasculitis Serv, Norwich, Norfolk, England

[21] Univ Florence, Meyer Children Hosp, Florence, Italy

[22] Univ Florence, Careggi Univ Hosp, Florence, Italy

[23] Azienda USL IRCCS Reggio Emilia, Unit Rheumatol, Reggio Emilia, Italy

[24] Univ Modena & Reggio Emilia, Dept Surg Med Dent & Morphol Sci Interest Trans, Modena, Italy

[25] Hannover Med Sch, Dept Resp Med, Hannover, Germany

[26] Hannover Med Sch, German Ctr Lung Res, Hannover, Germany

[27] Univ Milano Bicocca, Dept Med & Surg, Milan, Italy

[28] ASST Monza, Renal Unit, Monza, Italy

[29] Univ Paris Cite, Bichat Hosp, Reference Ctr Rare Resp Dis, AP HP Nord, Paris, France

[30] Univ Paris, Hop Cochin, Serv Med Interne, Paris, France

[31] Univ Freiburg, Univ Med Ctr Freiburg, Fac Med, Clin Rheumatol & Clin Immunol, Freiburg, Germany

[32] Univ Crete, Rheumatol Clin Immunol & Allergy, Sch Med, Iraklion, Greece

[33] Inst Mol Biol & Biotechnol, Lab Autoimmun Inflammat, Iraklion, Crete, Greece

[34] Univ Paris, Cochin Hosp, AP HP, Natl Referral Ctr Rare Syst Autoimmune Dis,Intern, Paris, France

[35] Univ Cambridge, Addenbrookes Hosp, Box 118, Cambridge, England

[36] Cambridge Univ Hosp NHS Fdn Trust, Cambridge, England

[37] Univ Florence, Dept Biomed Expt & Clin Sci Mario Serio, Florence, Italy

来源：

NATURE REVIEWS RHEUMATOLOGY | 2023年 / 19卷 / 06期

关键词：

CHURG-STRAUSS-SYNDROME; ANCA-ASSOCIATED VASCULITIS; ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES; SYSTEMIC-NECROTIZING-VASCULITIDES; VENOUS THROMBOEMBOLIC EVENTS; TERM-FOLLOW-UP; POLYARTERITIS-NODOSA; MICROSCOPIC POLYANGIITIS; WEGENERS-GRANULOMATOSIS; POOR-PROGNOSIS;

D O I：

10.1038/s41584-023-00958-w

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：378 / 393

页数：16

共 120 条

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