Original Articles
7 April 2025

Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies

Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
730
Views
282
Downloads

Authors

Objective. Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASS). Treatment typically involves high-dose corticosteroids (CCS) and conventional synthetic disease-modifying antirheumatic drugs (csDMARD). Rituximab (RTX) has shown effectiveness in refractory cases. Our real-life study aimed to assess the safety and effectiveness of RTX treatment in IIM patients.

Methods. We conducted a retrospective study including patients with IIM refractory to both high-dose CCS and csDMARD. Patients were treated with a full RTX dose (2 g every 6 months). Laboratory and clinical data, along with the total improvement score (TIS), were assessed to evaluate RTX effectiveness and safety. Data were analyzed using GraphPad Prism (v. 9.5.1).

Results. A total of 41 patients received the full RTX dose (15 DM, 15 ASS, 5 PM, and 6 IMNM). This treatment regimen significantly reduced daily CCS usage from 15 mg [interquartile range (IQR) 12.5-25 mg] at baseline to 5 mg (IQR 5-5 mg) after 1 year of treatment (p<0.001). Additionally, over 90% of patients achieved at least a minimal TIS at 12 months, which was maintained at 24 months. At 1 year, RTX persistence was 68.3%. Although reductions in serum immunoglobulins (Ig)A and IgM levels were observed, no cases of severe hypogammaglobulinemia (IgG<400 mg/dL) occurred. The most common reason for treatment interruption was adverse skin reaction (6 cases) during RTX infusion, while infections involved most frequently the respiratory tract (5 cases).

Conclusions. RTX demonstrated effectiveness in various subsets of IIMs, often leading to clinical improvement and significantly reducing the CCS dose.

Altmetrics

Downloads

Download data is not yet available.

Citations

Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Stine LC, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primers 2021; 7: 86. DOI: https://doi.org/10.1038/s41572-021-00321-x
Rizzo C, La Barbera L, Barletta G, Camarda F, Donzella D, Romano G, et al. Characterising oesophageal motility disorders by high-resolution impedance manometry in dermatomyositis patients. Clin Exp Rheumatol 2024; 42: 344-50. DOI: https://doi.org/10.55563/clinexprheumatol/h4drr2
Basnayake C, Cash K, Blumbergs P, Limaye V. Use of rituximab in histologically confirmed idiopathic inflammatory myositis: a case series. Clin Rheumatol 2015; 34: 371-7. DOI: https://doi.org/10.1007/s10067-013-2449-2
Nalotto L, Iaccarino L, Zen M, Gatto M, Borella W, Domeneghetti M, et al. Rituximab in refractory idiopathic inflammatory myopathies and antisynthetase syndrome: personal experience and review of the literature. Immunol Res 2013; 56: 362-70. DOI: https://doi.org/10.1007/s12026-013-8408-9
Mahler EAM, Blom M, Voermans NC, Van Engelen BGM, Van Riel PLCM, Vonk MC. Rituximab treatment in patients with refractory inflammatory myopathies. Rheumatology 2011; 50: 2206-13. DOI: https://doi.org/10.1093/rheumatology/ker088
Oldroyd AGS, Lilleker JB, Amin T, Aragon O, Bechman K, Cuthbert V, et al. British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy. Rheumatology 2022; 61: 1760-8. DOI: https://doi.org/10.1093/rheumatology/keac115
Glaubitz S, Zeng R, Schmidt J. New insights into the treatment of myositis. Ther Adv Musculoskelet Dis 2020; 12: 1759720X1988649. DOI: https://doi.org/10.1177/1759720X19886494
Grazzini S, Rizzo C, Conticini E, D'Alessandro R, La Barbera L, D'Alessandro M, et al. The role of bDMARDs in idiopathic inflammatory myopathies: a systematic literature review. Autoimmun Rev 2023; 22: 103264. DOI: https://doi.org/10.1016/j.autrev.2022.103264
Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C. Efficacy and safety of rituximab treatment in patients with idiopathic inflammatory myopathies: a systematic review and meta-analysis. Front Immunol 2022; 13: 1051609. DOI: https://doi.org/10.3389/fimmu.2022.1051609
Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo‐phase trial. Arthritis Rheum 2013; 65: 314-24. DOI: https://doi.org/10.1002/art.37754
Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis 2017; 76: 1955-64. DOI: https://doi.org/10.1136/annrheumdis-2017-212786
Allenbach Y, Mammen AL, Benveniste O, Stenzel W, Immune-mediated necrotizing myopathies working group. 224th ENMC international workshop:: clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14-16 October 2016. Neuromuscul Disord 2018; 28: 87-99. DOI: https://doi.org/10.1016/j.nmd.2017.09.016
Boumaza X, Lafaurie M, Treiner E, Walter O, Pugnet G, Martin-Blonderl G, et al. Infectious risk when prescribing rituximab in patients with hypogammaglobulinemia acquired in the setting of autoimmune diseases. Int Immunopharmacol 2023; 120: 110342. DOI: https://doi.org/10.1016/j.intimp.2023.110342
Saygin D, Kim H, Douglas C, Erman B, Wilkerson J, McGrath JA, et al. Performance of the 2016 ACR-EULAR Myositis Response Criteria in adult dermatomyositis/polymyositis therapeutic trials and consensus profiles. Rheumatology 2023; 62: 3672-9. DOI: https://doi.org/10.1093/rheumatology/kead110
Barsotti S, Lundberg IE. Current treatment for myositis. Curr Treatm Opt Rheum 2018; 4: 299-315. DOI: https://doi.org/10.1007/s40674-018-0106-2
Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum 2005; 52: 601-7. DOI: https://doi.org/10.1002/art.20849
McHugh NJ, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol 2018; 14: 290-302. DOI: https://doi.org/10.1038/nrrheum.2018.56
Aggarwal R, Bandos A, Reed AM, Ascherman DP, Barohn RJ, Feldman BM, et al. Predictors of clinical improvement in rituximab-treated refractory adult and juvenile dermatomyositis and adult polymyositis. Arthritis Rheumatol 2014; 66: 740-9. DOI: https://doi.org/10.1002/art.38270
De Souza FHC, Miossi R, De Moraes JCB, Bonfá E, Shinjo SK. Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies. Adv Rheumatol 2018; 58: 31. DOI: https://doi.org/10.1186/s42358-018-0030-z
Marie I, Dominique S, Janvresse A, Levesque H, Menard JF. Rituximab therapy for refractory interstitial lung disease related to antisynthetase syndrome. Respir Med 2012; 106: 581-7. DOI: https://doi.org/10.1016/j.rmed.2012.01.001
Fasano S, Gordon P, Hajji R, Loyo E, Isenberg DA. Rituximab in the treatment of inflammatory myopathies: a review. Rheumatology 2017; 56: 26-36. DOI: https://doi.org/10.1093/rheumatology/kew146
Ahn GY, Suh CH, Kim YG, Park YB, Shim SC, Lee SH, et al. Efficacy and safety of rituximab in korean patients with refractory inflammatory myopathies. J Korean Med Sci 2020; 35: e335. DOI: https://doi.org/10.3346/jkms.2020.35.e335
Xiong A, Yang G, Song Z, Xiong C, Liu D, Shuai Y, et al. Rituximab in the treatment of immune-mediated necrotizing myopathy: a review of case reports and case series. Ther Adv Neurol Disord 2021; 14: 1756286421998918. DOI: https://doi.org/10.1177/1756286421998918
Unger L, Kampf S, Lüthke K, Aringer M. Rituximab therapy in patients with refractory dermatomyositis or polymyositis: differential effects in a real-life population. Rheumatology 2014; 53: 1630-8. DOI: https://doi.org/10.1093/rheumatology/keu024
Santos VA, Aragón CC, Posso-Osorio I, Naranjo-Escobar J, Milisenda JC, Obando MA, et al. Rituximab for inflammatory myopathies in a Colombian cohort. J Clin Rheumatol 2021; 27: S232-5. DOI: https://doi.org/10.1097/RHU.0000000000001620
Barmettler S, Ong MS, Farmer JR, Choi H, Walter J. Association of immunoglobulin levels, infectious risk, and mortality with rituximab and hypogammaglobulinemia. JAMA Netw Open 2018; 1: e184169. DOI: https://doi.org/10.1001/jamanetworkopen.2018.4169
Singh N, Madhira V, Hu C, Olex AL, Bergquist T Fitzgerlad KC, et al. Rituximab is associated with worse COVID-19 outcomes in patients with rheumatoid arthritis: a retrospective, nationally sampled cohort study from the U.S. National COVID Cohort Collaborative (N3C). Semin Arthritis Rheum 2023; 58: 152149. DOI: https://doi.org/10.1016/j.semarthrit.2022.152149
Conticini E, d’Alessandro M, Grazzini S, Fornaro M, Sabella D, Lopalco G, et al. Relapses of idiopathic inflammatory myopathies after vaccination against COVID-19: a real-life multicenter Italian study. Intern Emerg Med 2022; 17: 1921-8. DOI: https://doi.org/10.1007/s11739-022-03028-3
Furer V, Rondaan C, Heijstek MW, Agmon-Levin N, van Assen S, Bijl M, et al. 2019 update of EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis 2020; 79: 39-52. DOI: https://doi.org/10.1136/annrheumdis-2019-215882
Marco H, Smith RM, Jones RB, Guerry MJ, Catapano F, Burns S, et al. The effect of rituximab therapy on immunoglobulin levels in patients with multisystem autoimmune disease. BMC Musculoskelet Disord 2014; 15: 178. DOI: https://doi.org/10.1186/1471-2474-15-178
Evangelatos G, Fragoulis GE, Klavdianou K, Moschopoulou M, Vassilopoulos D, Iliopoulos A. Hypogammaglobulinemia after rituximab for rheumatoid arthritis is not rare and is related with good response: 13 years real-life experience. Rheumatology 2021; 60: 2375-82. DOI: https://doi.org/10.1093/rheumatology/keaa617
Conticini E, d’Alessandro M, Grazzini S, Rizzo C, Fornaro M, Cameli P, et al. Rituximab-induced hypogammaglobulinaemia in patients affected by idiopathic inflammatory myopathies: a multicentre study. Clin Exp Rheumatol 2023; 41: 285-90. DOI: https://doi.org/10.55563/clinexprheumatol/790ihy
Chinoy H, Lilleker JB. Pitfalls in the diagnosis of myositis. Best Pract Res Clin Rheumatol 2020; 34: 101486. DOI: https://doi.org/10.1016/j.berh.2020.101486
Amato AA, Griggs RC. Unicorns, dragons, polymyositis, and other mythological beasts. Neurology 2003; 61: 288-9. DOI: https://doi.org/10.1212/WNL.61.3.288

How to Cite



Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies. (2025). Reumatismo. https://doi.org/10.4081/reumatismo.2025.1817