PMID- 36283646
OWN - NLM
STAT- MEDLINE
DCOM- 20221227
LR  - 20221230
IS  - 1873-0183 (Electronic)
IS  - 1568-9972 (Linking)
VI  - 22
IP  - 1
DP  - 2023 Jan
TI  - Eosinophilic granulomatosis with polyangiitis: A review.
PG  - 103219
LID - S1568-9972(22)00189-6 [pii]
LID - 10.1016/j.autrev.2022.103219 [doi]
AB  - Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multi-system, 
      inflammatory disease, belonging to the group of anti-neutrophil cytoplasmic 
      antibody (ANCA)-associated vasculitides (AAV). Previously known as Churg-Strauss 
      syndrome, EGPA is characterised by late-onset asthma, eosinophilia and vasculitis 
      affecting small-to-medium vessels. This disease behaves differently in many 
      aspects to the other AAV and is often excluded from AAV studies. The disease is 
      poorly understood and, due to it rarity and unique manifestations, there has been 
      limited research progress to optimise our understanding of its complex 
      pathogenesis and ability to develop management options - although the success of 
      interleukin-5 inhibitors such as Mepolizumab has been a welcome development. The 
      pathophysiology also appears to be different to other forms of AAV and hence 
      management strategies that work for AAV may not fully apply to this condition. 
      There is no current standard therapy for EGPA although corticosteroids are almost 
      universally used for treatment alongside other agents and encouraging modes of 
      treatment continue to evolve beyond glucocorticoid immunosuppression (including 
      interleukin-5 inhibition). There is therefore a significant ongoing unmet need 
      for efficacious steroid-sparing immunosuppressing agents. The prognosis also 
      diverges from other forms of AAV, and we discuss the pathophysiology, clinical 
      features and diagnosis, management and prognosis in this article.
CI  - Copyright (c) 2022 Elsevier B.V. All rights reserved.
FAU - White, Jpe
AU  - White J
AD  - St George's Hospital, Blackshaw Road, Tooting, London SW17 0QT, United Kingdom.
FAU - Dubey, S
AU  - Dubey S
AD  - Dept of Rheumatology, Oxford University Hospitals NHS FT, Windmill Road, Oxford 
      OX3 7LD, United Kingdom; Nuffield Department of Orthopaedics, Rheumatology and 
      Musculoskeletal Sciences, University of Oxford, Windmill Road, Oxford OX3 7HE, 
      United Kingdom. Electronic address: Shirish.dubey@ouh.nhs.uk.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20221022
PL  - Netherlands
TA  - Autoimmun Rev
JT  - Autoimmunity reviews
JID - 101128967
RN  - 0 (Interleukin-5)
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
SB  - IM
MH  - Humans
MH  - *Churg-Strauss Syndrome/therapy/drug therapy
MH  - *Granulomatosis with Polyangiitis/diagnosis/drug therapy
MH  - Interleukin-5/therapeutic use
MH  - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
MH  - Prognosis
MH  - Antibodies, Antineutrophil Cytoplasmic
OTO - NOTNLM
OT  - ANCA-associated vasculitis
OT  - Asthma
OT  - Churg-Strauss syndrome
OT  - Eosinophilia
OT  - Eosinophilic granulomatosis with polyangiitis
OT  - Eosinophilic vasculitis
OT  - Vasculitis
COIS- Declaration of Competing Interest The authors declare that they have no known 
      competing financial interests or personal relationships that could have appeared 
      to influence the work reported in this paper.
EDAT- 2022/10/26 06:00
MHDA- 2022/12/28 06:00
CRDT- 2022/10/25 19:28
PHST- 2022/10/02 00:00 [received]
PHST- 2022/10/19 00:00 [accepted]
PHST- 2022/10/26 06:00 [pubmed]
PHST- 2022/12/28 06:00 [medline]
PHST- 2022/10/25 19:28 [entrez]
AID - S1568-9972(22)00189-6 [pii]
AID - 10.1016/j.autrev.2022.103219 [doi]
PST - ppublish
SO  - Autoimmun Rev. 2023 Jan;22(1):103219. doi: 10.1016/j.autrev.2022.103219. Epub 
      2022 Oct 22.