PMID- 26506114 OWN - NLM STAT- MEDLINE DCOM- 20160614 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 15 IP - 2 DP - 2016 Feb TI - Dividing the Janus vasculitis? Pathophysiology of eosinophilic granulomatosis with polyangitis. PG - 139-45 LID - S1568-9972(15)00218-9 [pii] LID - 10.1016/j.autrev.2015.10.006 [doi] AB - Eosinophilic granulomatosis with polyangitis (EGPA) is a rare small- and medium-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). It is commonly divided into two phenotypes depending on the presence of ANCAs targeting myeloperoxidase (MPO). MPO-ANCAs are present in 31% to 38% of patients and are associated with a vasculitis phenotype of the disease, whereas patients without MPO-ANCA are at risk of cardiac involvement. Despite significant advances in understanding the overall pathogenesis of the disease, the explanation for this dichotomy is still unclear. In this review, we synthesize our knowledge of the pathogenesis of EGPA and attempt to i) distinguish EGPA from other diseases including other AAVs, asthma, allergy and hypereosinophilic-associated conditions and ii) speculate about the preponderant mechanisms, which could explain the two disease phenotypes. CI - Copyright (c) 2015 Elsevier B.V. All rights reserved. FAU - Chaigne, Benjamin AU - Chaigne B AD - INSERM, U1016, Institut Cochin, Paris, France; CNRS, UMR8104, Paris, France; Universite Paris Descartes, Sorbonne Paris Cite, Paris, France; Universite Paris Descartes, Faculte de Medecine, Pole de Medecine Interne, Centre de reference pour les vascularites necrosantes et la sclerodermie systemique, hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris, France. FAU - Terrier, Benjamin AU - Terrier B AD - INSERM, U1016, Institut Cochin, Paris, France; CNRS, UMR8104, Paris, France; Universite Paris Descartes, Sorbonne Paris Cite, Paris, France; Universite Paris Descartes, Faculte de Medecine, Pole de Medecine Interne, Centre de reference pour les vascularites necrosantes et la sclerodermie systemique, hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris, France. FAU - Thieblemont, Nathalie AU - Thieblemont N AD - INSERM, U1016, Institut Cochin, Paris, France; CNRS, UMR8104, Paris, France; Universite Paris Descartes, Sorbonne Paris Cite, Paris, France. FAU - Witko-Sarsat, Veronique AU - Witko-Sarsat V AD - INSERM, U1016, Institut Cochin, Paris, France; CNRS, UMR8104, Paris, France; Universite Paris Descartes, Sorbonne Paris Cite, Paris, France. FAU - Mouthon, Luc AU - Mouthon L AD - INSERM, U1016, Institut Cochin, Paris, France; CNRS, UMR8104, Paris, France; Universite Paris Descartes, Sorbonne Paris Cite, Paris, France; Universite Paris Descartes, Faculte de Medecine, Pole de Medecine Interne, Centre de reference pour les vascularites necrosantes et la sclerodermie systemique, hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris, France. Electronic address: luc.mouthon@cch.aphp.fr. LA - eng PT - Journal Article PT - Review DEP - 20151023 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - EC 1.11.1.7 (Peroxidase) SB - IM MH - Animals MH - Antibodies, Antineutrophil Cytoplasmic/immunology MH - Asthma/complications MH - B-Lymphocytes/immunology MH - Eosinophils/*immunology MH - Granulomatosis with Polyangiitis/complications/*immunology MH - Humans MH - Peroxidase/immunology OTO - NOTNLM OT - ANCA-MPO OT - Eosinophilia OT - Eosinophilic granulomatosis with polyangitis OT - Th2 response OT - Vasculitis EDAT- 2015/10/28 06:00 MHDA- 2016/06/15 06:00 CRDT- 2015/10/28 06:00 PHST- 2015/10/02 00:00 [received] PHST- 2015/10/15 00:00 [accepted] PHST- 2015/10/28 06:00 [entrez] PHST- 2015/10/28 06:00 [pubmed] PHST- 2016/06/15 06:00 [medline] AID - S1568-9972(15)00218-9 [pii] AID - 10.1016/j.autrev.2015.10.006 [doi] PST - ppublish SO - Autoimmun Rev. 2016 Feb;15(2):139-45. doi: 10.1016/j.autrev.2015.10.006. Epub 2015 Oct 23.