PMID- 34244040 OWN - NLM STAT- MEDLINE DCOM- 20220215 LR - 20220215 IS - 1424-3911 (Electronic) IS - 1424-3903 (Linking) VI - 21 IP - 7 DP - 2021 Oct TI - Efficacy and safety of rituximab for IgG4-related pancreato-biliary disease: A systematic review and meta-analysis. PG - 1395-1401 LID - S1424-3903(21)00494-4 [pii] LID - 10.1016/j.pan.2021.06.009 [doi] AB - BACKGROUND: Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC) belong to the IgG4-related disease (IgG4-RD) spectrum. Both entities respond to glucocorticoids, but iatrogenic toxicity associated with prolonged steroid therapy and relapse represent relevant clinical concerns in the long-term. Rituximab is increasingly used as an effective alternative strategy to induce remission but data regarding the safety and efficacy of B-cell depletion therapy for pancreato-biliary involvement of IgG4-RD are limited. We performed a systematic review and meta-analysis to estimate the rate of remission, flare, and adverse events (AEs) occurring in pancreato-biliary IgG4-RD following rituximab treatment. METHODS: The MEDLINE, SCOPUS, and EMBASE databases were searched from inception to December 2020 to identify studies reporting the outcomes of IgG4-related pancreato-biliary disease after treatment with rituximab. Studies involving >/=2 patients were selected. In case of duplicated studies, the most recent or the one with the biggest N were chosen. The study was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Pooled effects were calculated using a random-effect model and expressed in terms of pooled remission, relapse, and AEs rates. RESULTS: Seven cohort studies met inclusion criteria and 101 patients were included. Reasons for rituximab administration were new disease onset (18.5%), disease flare after glucocorticoids (63.5%), and glucocorticoids intolerance (17.9%). The median follow-up time was 19 months. The pooled rate of complete response at 6 months was 88.9% (95%CI 80.5-93.9) with no heterogeneity (I(2) = 0%). The pooled estimate of relapse rate was 21% (95%CI 10.5-40.3) with moderate heterogeneity (I(2) = 51%). A higher rate of relapse (35.9%, 95%CI 17.3-60.1) was reported in studies including patients with multiorgan involvement (OOI). The median time to relapse was 10 months. The pooled estimate of rituximab-related AEs was 25% (95%CI 8.8-53) with substantial heterogeneity (I(2) = 73.6%). No publication bias was observed. CONCLUSION: Treatment of IgG4-related pancreato-biliary disease with rituximab is associated with high remission rate, a higher relapse rate in the presence of OOI, and limited AEs. Randomized controlled trials with adequate power are needed to confirm these findings. CI - Copyright (c) 2021 IAP and EPC. Published by Elsevier B.V. All rights reserved. FAU - Lanzillotta, Marco AU - Lanzillotta M AD - Universita Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: lanzillotta.marco@hsr.it. FAU - Della-Torre, Emanuel AU - Della-Torre E AD - Universita Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy. FAU - Wallace, Zachary S AU - Wallace ZS AD - Rheumatology Unit, Massachusetts General Hospital, Boston, MA, USA. FAU - Stone, John H AU - Stone JH AD - Rheumatology Unit, Massachusetts General Hospital, Boston, MA, USA. FAU - Karadag, Omer AU - Karadag O AD - Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Hacettepe University, Sihhiye-Ankara, Turkey. FAU - Fernandez-Codina, Andreu AU - Fernandez-Codina A AD - Rheumatology Division and General Internal Medicine division-Windsor Campus, Western University, 268 Grosvenor St, D2-191, Rheumatology Centre, St. Joseph's Health Care, London, Ontario, Canada. FAU - Arcidiacono, Paolo Giorgio AU - Arcidiacono PG AD - Division of Pancreatic Surgery and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy. FAU - Falconi, Massimo AU - Falconi M AD - Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - Universita Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy. FAU - Capurso, Gabriele AU - Capurso G AD - Division of Pancreatic Surgery and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20210703 PL - Switzerland TA - Pancreatology JT - Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] JID - 100966936 RN - 0 (Glucocorticoids) RN - 0 (Immunoglobulin G) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Glucocorticoids MH - Humans MH - Immunoglobulin G MH - *Immunoglobulin G4-Related Disease/drug therapy MH - Recurrence MH - Rituximab/adverse effects OTO - NOTNLM OT - Autoimmune pancreatitis OT - IgG4-related disease OT - Rituximab -meta-analysis EDAT- 2021/07/11 06:00 MHDA- 2022/02/16 06:00 CRDT- 2021/07/10 05:35 PHST- 2021/05/11 00:00 [received] PHST- 2021/06/22 00:00 [revised] PHST- 2021/06/29 00:00 [accepted] PHST- 2021/07/11 06:00 [pubmed] PHST- 2022/02/16 06:00 [medline] PHST- 2021/07/10 05:35 [entrez] AID - S1424-3903(21)00494-4 [pii] AID - 10.1016/j.pan.2021.06.009 [doi] PST - ppublish SO - Pancreatology. 2021 Oct;21(7):1395-1401. doi: 10.1016/j.pan.2021.06.009. Epub 2021 Jul 3.