Respiratory investigation · 2026
Nintedanib for non-IPF progressive pulmonary fibrosis: A systematic review and narrative synthesis of real-world effectiveness and safety.
СтатьяСистематический обзорНазвание и аннотация: Перевод в очереди
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Srivali Narat, De Giacomi Federica
Аннотация
BACKGROUND: Progressive pulmonary fibrosis (PPF) encompasses non-IPF fibrosing interstitial lung diseases that progress despite conventional management. Nintedanib is the only licensed antifibrotic for non-IPF PPF, yet real-world populations differ substantially from INBUILD trial participants, particularly in near-universal concomitant immunosuppressive therapy (IST) use.
METHODS: A systematic search of PubMed/MEDLINE, EMBASE, and Cochrane Library (inception-May 2026) identified observational studies, registries, and open-label trial extensions evaluating antifibrotics in adults with non-IPF PPF. Studies reporting lung function, disease progression, or safety with ≥6 months follow-up were eligible. Data were narratively synthesized per PRISMA 2020 and SWiM guidelines; formal meta-analysis was precluded by substantial heterogeneity. Risk of bias and certainty of evidence were assessed using ROBINS-I and GRADE.
RESULTS: Ten studies were included (N = 3228; 9 countries; 2014-2025). Real-world patients showed greater disease severity at initiation, with 55-100% receiving concomitant IST. Nintedanib was associated with attenuated FVC decline in uncontrolled pre-post analyses (-72 to -89 mL/year on treatment vs. -188 to -240 mL/year pre-treatment); these estimates are hypothesis-generating only. Diarrhea occurred in 29-77%; permanent discontinuation in 9-29%. Nintedanib combined with mycophenolate, rituximab, or tocilizumab was not associated with excess serious adverse events. Pirfenidone evidence comprised a single 11-patient case series.
CONCLUSIONS: Very low-certainty real-world evidence suggests nintedanib may attenuate disease progression and is generally tolerable across diverse ILD subtypes and IST backgrounds. Pirfenidone evidence remains insufficient. Prospective controlled studies and registries examining antifibrotic-IST combinations and long-term patient-centered outcomes are an urgent priority.
METHODS: A systematic search of PubMed/MEDLINE, EMBASE, and Cochrane Library (inception-May 2026) identified observational studies, registries, and open-label trial extensions evaluating antifibrotics in adults with non-IPF PPF. Studies reporting lung function, disease progression, or safety with ≥6 months follow-up were eligible. Data were narratively synthesized per PRISMA 2020 and SWiM guidelines; formal meta-analysis was precluded by substantial heterogeneity. Risk of bias and certainty of evidence were assessed using ROBINS-I and GRADE.
RESULTS: Ten studies were included (N = 3228; 9 countries; 2014-2025). Real-world patients showed greater disease severity at initiation, with 55-100% receiving concomitant IST. Nintedanib was associated with attenuated FVC decline in uncontrolled pre-post analyses (-72 to -89 mL/year on treatment vs. -188 to -240 mL/year pre-treatment); these estimates are hypothesis-generating only. Diarrhea occurred in 29-77%; permanent discontinuation in 9-29%. Nintedanib combined with mycophenolate, rituximab, or tocilizumab was not associated with excess serious adverse events. Pirfenidone evidence comprised a single 11-patient case series.
CONCLUSIONS: Very low-certainty real-world evidence suggests nintedanib may attenuate disease progression and is generally tolerable across diverse ILD subtypes and IST backgrounds. Pirfenidone evidence remains insufficient. Prospective controlled studies and registries examining antifibrotic-IST combinations and long-term patient-centered outcomes are an urgent priority.