Background and objective <p>Currently, there are only a few effective treatments for anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) positive dermatomyositis (DM)-associated interstitial lung disease (ILD). The aim of this study was to evaluate the efficacy and safety of baricitinib in patients with MDA5 + DM-ILD.</p> Methods <p>The study was a retrospective cohort design that evaluated patients with MDA5 + DM-ILD who had received or not received baricitinib. Clinical symptoms, laboratory data, and survival were compared in the two groups.</p> Results <p>Thirty-nine patients with MDA5 + DM-ILD who received baricitinib were included in the study. After baricitinib therapy, 31 patients (79.5%) had a clinical improvement that included Gottron’s sign (<i>p</i> &lt; 0.001), heliotrope rash (<i>p</i> &lt; 0.001), and dyspnea (<i>p</i> = 0.003). The median HRCT score decreased from 115 to 97.5 (<i>p</i> &lt; 0.001), while the FVC improved significantly (76.44% vs. 90.73%, <i>p</i> &lt; 0.001). In addition, serum ferritin levels (739.9 ng/ml vs. 56.3 ng/ml, <i>p</i> &lt; 0.001) and lactate dehydrogenase (LDH) levels (313 U/L vs. 233 U/L, <i>p</i> = 0.003) decreased significantly. Notably, lymphocyte counts increased significantly, CD4 + T cells (354 cells/µl vs. 663 cells/µl, <i>p</i> &lt; 0.001), and CD8 + T cells (180 cells/µl vs. 449 cells/µl, <i>p</i> &lt; 0.001). Furthermore, the glucocorticoid dose reduced significantly from 40&#xa0;mg daily to 10&#xa0;mg daily (<i>p</i> &lt; 0.001). Compared with the control group, the baricitinib group had a greater six-month survival (87.2% vs. 70%, <i>p</i> = 0.047). While first-line baricitinib showed a superior survival rate compared to conventional therapy (84.6% vs. 52.9%, <i>p</i> = 0.046).</p> Conclusion <p>This study indicates that Baricitinib is a potential therapeutic for MDA5 + DM-ILD, reducing LDH and ferritin, ameliorating ILD progression, and improving survival, with first-line use may provide a significant survival advantage. However, larger prospective controlled studies are required to evaluate its efficacy.</p>

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Baricitinib could improve the prognosis of anti-MDA5 antibody positive dermatomyositis associated interstitial lung disease

  • Shuai Zhu,
  • Shanshan Li,
  • Lipu Shi,
  • Tianshu Chu,
  • Zhenguo Huang,
  • Xin Lu,
  • Guochun Wang,
  • Yongpeng Ge

摘要

Background and objective

Currently, there are only a few effective treatments for anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) positive dermatomyositis (DM)-associated interstitial lung disease (ILD). The aim of this study was to evaluate the efficacy and safety of baricitinib in patients with MDA5 + DM-ILD.

Methods

The study was a retrospective cohort design that evaluated patients with MDA5 + DM-ILD who had received or not received baricitinib. Clinical symptoms, laboratory data, and survival were compared in the two groups.

Results

Thirty-nine patients with MDA5 + DM-ILD who received baricitinib were included in the study. After baricitinib therapy, 31 patients (79.5%) had a clinical improvement that included Gottron’s sign (p < 0.001), heliotrope rash (p < 0.001), and dyspnea (p = 0.003). The median HRCT score decreased from 115 to 97.5 (p < 0.001), while the FVC improved significantly (76.44% vs. 90.73%, p < 0.001). In addition, serum ferritin levels (739.9 ng/ml vs. 56.3 ng/ml, p < 0.001) and lactate dehydrogenase (LDH) levels (313 U/L vs. 233 U/L, p = 0.003) decreased significantly. Notably, lymphocyte counts increased significantly, CD4 + T cells (354 cells/µl vs. 663 cells/µl, p < 0.001), and CD8 + T cells (180 cells/µl vs. 449 cells/µl, p < 0.001). Furthermore, the glucocorticoid dose reduced significantly from 40 mg daily to 10 mg daily (p < 0.001). Compared with the control group, the baricitinib group had a greater six-month survival (87.2% vs. 70%, p = 0.047). While first-line baricitinib showed a superior survival rate compared to conventional therapy (84.6% vs. 52.9%, p = 0.046).

Conclusion

This study indicates that Baricitinib is a potential therapeutic for MDA5 + DM-ILD, reducing LDH and ferritin, ameliorating ILD progression, and improving survival, with first-line use may provide a significant survival advantage. However, larger prospective controlled studies are required to evaluate its efficacy.