PMID- 36102147 OWN - NLM STAT- MEDLINE DCOM- 20221021 LR - 20221216 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 18 IP - 11 DP - 2022 Nov TI - How best to manage relapse and remission in ANCA-associated vasculitis. PG - 1135-1143 LID - 10.1080/1744666X.2022.2122954 [doi] AB - INTRODUCTION: A two-stage therapeutic approach is now applied as standard-of-care to treat antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs): first, glucocorticoids (GCs) combined with cyclophosphamide (CYC) or rituximab (RTX) to induce remission, and then relapse prevention with remission-maintenance therapy. Nonetheless, a substantial risk of relapse persists. AREAS COVERED: The authors provide an overview of the current state of AAV remission-induction after relapse and maintenance therapies, and discuss new strategies recommended to prevent and treat relapses, focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). EXPERT OPINION: For remission-induction after GPA or MPA relapse with organ-threatening manifestations, reintroduction or intensification of GCs in combination with CYC or RTX cycle is recommended; we prefer RTX in light of its superior responses obtained in patients with relapsing disease. Rapid tapering of GCs has been shown not to alter AAV evolution while decreasing the risk of serious infections. In contrast, for non-severe, active MPA, we recommend GCs alone as first-line therapy. For patients whose MPA remains uncontrolled by GCs alone, immunosuppressant adjunction can be a GC-sparing option or to counter GC intolerance. Once remission is achieved, we recommend prolonged maintenance therapy with preemptive low-dose (500 mg) RTX infusion biannually. FAU - Puechal, Xavier AU - Puechal X AUID- ORCID: 0000-0003-3573-9203 AD - National Referral Center for Rare Systemic Autoimmune Diseases, Hopital Cochin, Assistance Publique-Hopitaux de Paris (APHP) centre, Universite Paris Cite, Paris, France. AD - French Vasculitis Study Group, Hopital Cochin, Paris, France. FAU - Guillevin, Loic AU - Guillevin L AUID- ORCID: 0000-0002-4518-7345 AD - National Referral Center for Rare Systemic Autoimmune Diseases, Hopital Cochin, Assistance Publique-Hopitaux de Paris (APHP) centre, Universite Paris Cite, Paris, France. AD - French Vasculitis Study Group, Hopital Cochin, Paris, France. LA - eng PT - Journal Article DEP - 20220914 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 RN - 4F4X42SYQ6 (Rituximab) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 8N3DW7272P (Cyclophosphamide) RN - 0 (Immunosuppressive Agents) RN - 0 (Glucocorticoids) SB - IM MH - Humans MH - Rituximab/therapeutic use MH - Antibodies, Antineutrophil Cytoplasmic MH - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy MH - *Microscopic Polyangiitis/drug therapy MH - Remission Induction MH - Cyclophosphamide/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Recurrence MH - Glucocorticoids/therapeutic use MH - *Granulomatosis with Polyangiitis/drug therapy MH - Treatment Outcome OTO - NOTNLM OT - Cyclophosphamide OT - granulomatosis with polyangiitis OT - induction treatment OT - maintenance therapy OT - microscopic polyangiitis OT - plasma exchange OT - prognostic factors OT - relapse OT - rituximab EDAT- 2022/09/15 06:00 MHDA- 2022/10/22 06:00 CRDT- 2022/09/14 04:32 PHST- 2022/09/15 06:00 [pubmed] PHST- 2022/10/22 06:00 [medline] PHST- 2022/09/14 04:32 [entrez] AID - 10.1080/1744666X.2022.2122954 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2022 Nov;18(11):1135-1143. doi: 10.1080/1744666X.2022.2122954. Epub 2022 Sep 14.