Scholarlink

Introduction <p>Despite the established antifibrotic effects of nintedanib and pirfenidone, antifibrotic monotherapy remains insufficient for a subset of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD).</p> Methods <p>This retrospective case series analyzed 13 CTD-ILD patients who developed radiologic progression during pirfenidone or nintedanib monotherapy and were subsequently escalated to combination therapy with both agents. All patients received combination treatment for more than 6&#xa0;months at Tongji Hospital between July 2020 and November 2025. Clinical characteristics, HRCT findings, and outcomes were evaluated.</p> Results <p>Most patients demonstrated improvement or stabilization on lung HRCT under stable glucocorticoid and immunosuppressive therapy. Both the total HRCT (<i>β</i> = -0.140, SE = 0.054, <i>P</i> = 0.015) and consolidation score (<i>β</i> = -0.096, SE = 0.030, <i>P</i> = 0.003) showed significant decreases over time. Ground-glass opacities and consolidation were significantly reduced at 6 and 12&#xa0;months compared with baseline, respectively (all <i>P</i> &lt; 0.05). Reticular abnormalities, traction bronchiectasis, and honeycombing remained stable with a decreasing trend. Among the 13 patients, 8 (61.54%) reported adverse events (AEs), with no serious AEs or deaths observed.</p> Conclusion <p>In selected CTD-ILD patients with radiologic progression during antifibrotic monotherapy, combined pirfenidone and nintedanib therapy appeared feasible and generally tolerable. Some patients showed possible radiologic stabilization, although interpretation is limited by the retrospective uncontrolled design and small sample size.</p> Trial registration <p>Chinese Clinical Trial Register; <a href="https://www.chictr.org.cn/">https://www.chictr.org.cn/</a>; ChiCTR2500098990.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Pirfenidone combined with nintedanib in connective tissue disease – associated interstitial lung disease with radiologic progression during antifibrotic monotherapy: A case series

  • 期刊论文

  • 发表日期：

    2026年7月27日

查看全文
  • Xinyue Zhang,
  • Yuxue Chen,
  • Shaozhe Cai,
  • Cong Ye,
  • Ziwei Hu,
  • Ziyun Zhang,
  • Ying Wang,
  • Qiongjie Hu,
  • Lingli Dong

摘要

Introduction

Despite the established antifibrotic effects of nintedanib and pirfenidone, antifibrotic monotherapy remains insufficient for a subset of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD).

Methods

This retrospective case series analyzed 13 CTD-ILD patients who developed radiologic progression during pirfenidone or nintedanib monotherapy and were subsequently escalated to combination therapy with both agents. All patients received combination treatment for more than 6 months at Tongji Hospital between July 2020 and November 2025. Clinical characteristics, HRCT findings, and outcomes were evaluated.

Results

Most patients demonstrated improvement or stabilization on lung HRCT under stable glucocorticoid and immunosuppressive therapy. Both the total HRCT (β = -0.140, SE = 0.054, P = 0.015) and consolidation score (β = -0.096, SE = 0.030, P = 0.003) showed significant decreases over time. Ground-glass opacities and consolidation were significantly reduced at 6 and 12 months compared with baseline, respectively (all P < 0.05). Reticular abnormalities, traction bronchiectasis, and honeycombing remained stable with a decreasing trend. Among the 13 patients, 8 (61.54%) reported adverse events (AEs), with no serious AEs or deaths observed.

Conclusion

In selected CTD-ILD patients with radiologic progression during antifibrotic monotherapy, combined pirfenidone and nintedanib therapy appeared feasible and generally tolerable. Some patients showed possible radiologic stabilization, although interpretation is limited by the retrospective uncontrolled design and small sample size.

Trial registration

Chinese Clinical Trial Register; https://www.chictr.org.cn/; ChiCTR2500098990.