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

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...

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Main Authors: Stefano Stano, Fabio Cacciapaglia, Laura Coladonato, Giuseppe Lopalco, Florenzo Iannone, Marco Fornaro
Format: Article
Language:English
Published: PAGEPress Publications 2025-04-01
Series:Reumatismo
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Online Access:https://www.reumatismo.org/reuma/article/view/1817
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author Stefano Stano
Fabio Cacciapaglia
Laura Coladonato
Giuseppe Lopalco
Florenzo Iannone
Marco Fornaro
author_facet Stefano Stano
Fabio Cacciapaglia
Laura Coladonato
Giuseppe Lopalco
Florenzo Iannone
Marco Fornaro
author_sort Stefano Stano
collection DOAJ
description 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.
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spelling doaj-art-73735b04d1b746dd8b0adb5b74cd08f02025-08-20T02:16:19ZengPAGEPress PublicationsReumatismo0048-74492240-26832025-04-0110.4081/reumatismo.2025.1817Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathiesStefano Stano0Fabio Cacciapaglia1https://orcid.org/0000-0001-7479-4462Laura Coladonato2Giuseppe Lopalco3https://orcid.org/0000-0003-4524-9566Florenzo Iannone4Marco Fornaro5https://orcid.org/0000-0003-1716-7432Rheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of BariRheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of Bari; Department of Medicine and Surgery, LUM ‘G. De Gennaro’, CasamassimaRheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of BariRheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of BariRheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of BariRheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of Bari 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. https://www.reumatismo.org/reuma/article/view/1817Myositisrituximabsafetyeffectiveness
spellingShingle Stefano Stano
Fabio Cacciapaglia
Laura Coladonato
Giuseppe Lopalco
Florenzo Iannone
Marco Fornaro
Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
Reumatismo
Myositis
rituximab
safety
effectiveness
title Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
title_full Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
title_fullStr Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
title_full_unstemmed Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
title_short Real-life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
title_sort real life effectiveness of rituximab in different subsets of idiopathic inflammatory myopathies
topic Myositis
rituximab
safety
effectiveness
url https://www.reumatismo.org/reuma/article/view/1817
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