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Background <p>Idiopathic inflammatory myopathies (IIM) are a group of rare autoimmune disorders mainly involving skeletal muscles. Telitacicept is a BAFF/APRIL inhibitor, which has a promising application in B-cell-mediated autoimmune diseases; however, its efficacy in IIM patients is still unknown.</p> Methods <p>Retrospective analysis of 15 IIM patients treated with telitacicept for more than 6&#xa0;months in Xiangya Hospital between November 2023 and May 2025. Core clinical measures and glucocorticoid (GCS) dosage at baseline, 3&#xa0;months, 6&#xa0;months, and final follow-up were recorded. The treatment was evaluated according to the total improvement score (TIS) defined by the 2016 ACR/EULAR myositis response criteria.</p> Results <p>In the final follow-up, 11 patients (73.33%) had improved clinical symptoms after 8&#xa0;months of median treatment. Significant improvements were observed in physician/patient global assessments (PhGA/PtGA), manual muscle testing (MMT-8), health assessment questionnaire (HAQ) score, and creatine kinase (CK) levels (all <i>p</i> &lt; 0.05). The average GCS dose dropped from 23.67 ± 18.37 to 11.00 ± 11.05&#xa0;mg/d (<i>p</i> = 0.006). Serum IgG (<i>p</i> = 0.018), IgA (<i>p</i> &lt; 0.001), and IgM (<i>p</i> &lt; 0.001) decreased, while C3 (<i>p</i> = 0.001) and C4 (<i>p</i> = 0.030) increased. No serious drug-related adverse events occurred.</p> Conclusions <p>Telitacicept may be an effective and safety-promising therapeutic option for IIM, especially IMNM patients. Larger randomized controlled trials are required to confirm these findings.</p>

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A pilot study of telitacicept in predominantly immune-mediated necrotizing myopathy, retrospective analysis of a monocentric Chinese cohort

  • 期刊论文

  • 发表日期：

    2025年12月11日

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  • Wei Xiang,
  • Yijun Ren,
  • Huan Yang

摘要

Background

Idiopathic inflammatory myopathies (IIM) are a group of rare autoimmune disorders mainly involving skeletal muscles. Telitacicept is a BAFF/APRIL inhibitor, which has a promising application in B-cell-mediated autoimmune diseases; however, its efficacy in IIM patients is still unknown.

Methods

Retrospective analysis of 15 IIM patients treated with telitacicept for more than 6 months in Xiangya Hospital between November 2023 and May 2025. Core clinical measures and glucocorticoid (GCS) dosage at baseline, 3 months, 6 months, and final follow-up were recorded. The treatment was evaluated according to the total improvement score (TIS) defined by the 2016 ACR/EULAR myositis response criteria.

Results

In the final follow-up, 11 patients (73.33%) had improved clinical symptoms after 8 months of median treatment. Significant improvements were observed in physician/patient global assessments (PhGA/PtGA), manual muscle testing (MMT-8), health assessment questionnaire (HAQ) score, and creatine kinase (CK) levels (all p < 0.05). The average GCS dose dropped from 23.67 ± 18.37 to 11.00 ± 11.05 mg/d (p = 0.006). Serum IgG (p = 0.018), IgA (p < 0.001), and IgM (p < 0.001) decreased, while C3 (p = 0.001) and C4 (p = 0.030) increased. No serious drug-related adverse events occurred.

Conclusions

Telitacicept may be an effective and safety-promising therapeutic option for IIM, especially IMNM patients. Larger randomized controlled trials are required to confirm these findings.