☆ 4.7 Review

Efficacy of Pirfenidone and Nintedanib in Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis: A Systematic Review

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2023)

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Summary: The study suggests that pirfenidone may be less effective in uILD patients receiving concomitant MMF, while a beneficial treatment effect was observed in patients not receiving concomitant MMF regardless of previous corticosteroid use. Pirfenidone was well tolerated regardless of MMF and/or corticosteroid use.

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FRONTIERS IN MEDICINE (2022)

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Maria Molina-Molina et al.

Summary: The study analyzed patient baseline characteristics and compared the efficacy of pirfenidone versus placebo based on surgical lung biopsy (SLB) status, finding that pirfenidone may be an effective treatment for progressive fibrosing unclassifiable ILD over 24 weeks, regardless of SLB status. The differences in treatment effect between SLB subgroups suggest the need for further investigation based on pathological and radiological data. The comparison of baseline characteristics with other uILD populations highlighted the heterogeneity within these cohorts.

FRONTIERS IN MEDICINE (2022)

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Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial

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LANCET RESPIRATORY MEDICINE (2020)

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Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial

Athol U Wells et al.

Lancet Respiratory Medicine (2020)

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NEW ENGLAND JOURNAL OF MEDICINE (2018)

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An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis An Update of the 2011 Clinical Practice Guideline

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AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis

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Antifibrotic and Anti-inflammatory Activity of the Tyrosine Kinase Inhibitor Nintedanib in Experimental Models of Lung Fibrosis

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JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS (2014)

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An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias

William D. Travis et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2013)

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Pirfenidone for the treatment of Hermansky-Pudlak syndrome pulmonary fibrosis

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