Background/Purpose: Idiopathic inflammatory myopathies (IIM) are chronic inflammatory disorders that affect muscle, skin and lung to varying degrees. Interstitial lung disease (ILD) is a major cause of death. The clinical course, treatment response and prognosis of IIM-related ILD (IIM-ILD) is extremely heterogeneous, but in general, immunosuppressive therapy effectively prevents progression. Nonetheless, pulmonary fibrosis may gradually progress in some IIM-ILD patients despite optimal immunosuppressive therapy. Treatment with nintedanib, an anti-fibrotic agent, slows the rate of decline in forced vital capacity (FVC) in patients with progressive pulmonary fibrosis (PPF) accompanied by connective tissue disease, but its efficacy and safety for IIM-ILD has been unclear. Hence, the aim of the present study was to establish the efficacy and safety of nintedanib in patients with IIM-ILD.

Methods: Patients with stable IIM-ILD under immunosuppressive therapy seen at Tokai University from 2019 to 2023 were retrospectively evaluated for their clinical and immunological characteristics including underlying diagnosis, high resolution computed tomography (HRCT) findings, lung function test, treatment and prognosis. Patients were divided into two groups according to whether or not they received nintedanib in addition to immunosuppressive therapy. Comparisons between the two groups were made using the Chi-square test, Wilcoxon signed-rank test and Mann-Whitney test.

Results: Twenty-four patients with IIM-ILD participated in this study. Seven were treated with nintedanib (nintedanib group: male/female: 2/5, mean age ± SD: 64.7 ± 11.3, 4 (57%) had dermatomyositis (DM) and 3 (43%) anti-aminoacyl transfer RNA synthetase (ARS) syndrome); a further 17 patients received immunosuppressive therapy alone (non-nintedanib group: male/female: 8/9, mean age ± SD: 65.1 ± 10.0, 5 (29%) had polymyositis (PM), 6 (35%) had DM and 6 (35%) anti-ARS syndrome). There were no significant differences in frequencies of each HRCT pattern between groups (P=0.25). All patients in the nintedanib group had worsening diffuse fibrosis on HRCT before initiation of nintedanib therapy. In the longitudinal follow-up, the mean rate of FVC decline over 24 weeks in the nintedanib group was lower than in the non-nintedanib group although there was not significantly different, (-38.3 mL vs. -168.8 mL, P= 0.42). Adverse events (4 diarrhea, 3 appetite loss and one systemic edema) occurred in the nintedanib group, requiring dose reduction in 5 patients.

Conclusion: These results indicate that nintedanib slowed the decline in FVC and might be effective for reducing progression of fibrosis in patients with IIM-ILD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-and-safety-of-nintedanib-in-patients-with-idiopathic-inflammatory-myopathy-related-interstitial-lung-disease/