Original Article
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

https://doi.org/10.1016/j.ejim.2015.04.022Get rights and content
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Highlights

  • Consensus guidelines for EGPA diagnosis and management are still lacking.

  • Twenty-three disease experts used a modified Delphi process.

  • Twenty-two diagnostic, evaluation, and treatment recommendations were retained.

  • Thirteen high-priority research topics were also identified.

  • Regular updates will be necessary to maintain recommendation accuracy.

Abstract

Objective

To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) (EGPA).

Methods

The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached.

Results

Twenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed.

Discussion

These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.

Abbreviations

AAV
ANCA-associated vasculitides
ACR
American College of Rheumatology
ANCAs
antineutrophil cytoplasm antibodies
BVAS
Birmingham Vasculitis Activity Score
CRP
C-reactive protein
DVT
deep-vein thrombosis
ENT
ear, nose & throat
EGPA
eosinophilic granulomatosis with polyangiitis (Churg–Strauss)
ELISA
enzyme-linked immunosorbent assay
EULAR
European League Against Rheumatism
FFS
Five-Factor Score
GI
gastrointestinal
HE
hypereosinophilia
HES
hypereosinophilic syndromes
IL
interleukin
IV
intravenous
IVIg
IV immunoglobulins
LRA
leukotriene-receptor antagonists
MPO
myeloperoxidase
PR3
proteinase-3
SNV
systemic necrotizing vasculitis
VTEs
venous thromboembolic events

Keywords

Churg–Strauss syndrome
Anti-neutrophil cytoplasm antibody-associated vasculitis
Eosinophilia
Asthma
Recommendations
Eosinophilic granulomatosis with polyangiitis

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