ArticleClinical rheumatology2026
Pirfenidone combined with nintedanib in connective tissue disease - associated interstitial lung disease with radiologic progression during antifibrotic monotherapy: A case series.
Article in Clinical rheumatology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
introductionDespite 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).
methodsThis 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.
resultsMost 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.
conclusionIn 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 registrationChinese Clinical Trial Register; https://www.chictr.org.cn/ ; ChiCTR2500098990.
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