PMID- 32652104 OWN - NLM STAT- MEDLINE DCOM- 20201105 LR - 20201105 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 49 IP - 3 DP - 2020 Oct TI - Updates for the treatment of EGPA. PG - 104036 LID - S0755-4982(20)30024-5 [pii] LID - 10.1016/j.lpm.2020.104036 [doi] AB - Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is the least frequent antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). Major advances of our knowledge on its pathophysiology have revealed features of both AAV and eosinophilic disorders. The development of targeted biotherapies for both diseases opened new possibilities for EGPA management. In this review, we highlight the rationale underlying the routine treatment strategy, which relies mainly on corticosteroids, with immunosuppressant adjunction for severe disease. However, novel therapies are still needed for refractory/relapsing disease and to alleviate the corticosteroid-dependence of asthma and chronic rhinosinusitis. At present, the most promising biotherapies target either eosinophil biology, like mepolizumab, an anti-interleukin-5, or the B-cell compartment, with rituximab. Recent clinical data on new treatment options are discussed and therapeutic strategies are proposed. CI - Copyright (c) 2020 Elsevier Masson SAS. All rights reserved. FAU - Raffray, Loic AU - Raffray L AD - Department of Internal Medicine, Felix-Guyon University Hospital of La Reunion, CS11021, Saint Denis, Reunion. FAU - Guillevin, Loic AU - Guillevin L AD - Referral Center for Rare Systemic and Autoimmune Diseases, Department of Internal Medicine, Hopital Cochin, Universite Paris Descartes, 27, rue du Faubourg-Saint-Jacques, 75679 Paris Cedex 14, France. Electronic address: loic.guillevin@aphp.fr. LA - eng PT - Journal Article PT - Review DEP - 20200708 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 4F4X42SYQ6 (Rituximab) RN - 90Z2UF0E52 (mepolizumab) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Antibodies, Antineutrophil Cytoplasmic/adverse effects MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Biological Therapy/methods/trends MH - Churg-Strauss Syndrome/pathology/*therapy MH - Glucocorticoids/therapeutic use MH - Granulomatosis with Polyangiitis/pathology/therapy MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Rituximab/therapeutic use EDAT- 2020/07/12 06:00 MHDA- 2020/11/06 06:00 CRDT- 2020/07/12 06:00 PHST- 2019/08/25 00:00 [received] PHST- 2020/05/19 00:00 [revised] PHST- 2020/07/02 00:00 [accepted] PHST- 2020/07/12 06:00 [pubmed] PHST- 2020/11/06 06:00 [medline] PHST- 2020/07/12 06:00 [entrez] AID - S0755-4982(20)30024-5 [pii] AID - 10.1016/j.lpm.2020.104036 [doi] PST - ppublish SO - Presse Med. 2020 Oct;49(3):104036. doi: 10.1016/j.lpm.2020.104036. Epub 2020 Jul 8.