ArticleBMC pulmonary medicine2019
Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases.
Article in BMC pulmonary medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.
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Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- The Impact of Corticosteroids on Mortality in Acute Exacerbations of Idiopathic Pulmonary Fibrosis: A Meta-Analysis.Advances in respiratory medicine · 2025Pooled it
- Clinical significance of anti-neutrophil cytoplasmic antibody in idiopathic interstitial pneumonia: a retrospective observational study.BMC pulmonary medicine · 2025Observational
- Comparison of Six-minute Walk Test Parameters by Severity of Idiopathic and Non-idiopathic Pulmonary Fibrosis.Physical therapy research · 2025Article
- Incidence of antiretroviral therapy regimen modification and associated factors among People Living with HIV in Beijing, China.PLOS global public health · 2025Article
- Factors Affecting Best-Tolerated Dose of Pirfenidone in Patients with Fibrosing Interstitial Lung Disease.Journal of clinical medicine · 2023Article
- Safety and accuracy of transbronchial lung cryobiopsy in diagnosing desquamative interstitial pneumonia.The clinical respiratory journal · 2022Article
- Irreversibility of Pulmonary Fibrosis.Aging and disease · 2022Review
- The use of exhaled air analysis in discriminating interstitial lung diseases: a pilot study.Respiratory research · 2022Observational
- Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis.Orphanet journal of rare diseases · 2021Article
- Impact of bronchoalveolar lavage lymphocytosis on the effects of anti-inflammatory therapy in idiopathic non-specific interstitial pneumonia, idiopathic pleuroparenchymal fibroelastosis, and unclassifiable idiopathic interstitial pneumonia.Respiratory research · 2021Article
- Outcomes and Incidence of PF-ILD According to Different Definitions in a Real-World Setting.Frontiers in pharmacology · 2021Article
- Efficacy and safety of mycophenolate mofetil in the treatment of rheumatic disease-related interstitial lung disease: a narrative review.Drugs in context · 2021Review
- The natural history of progressive fibrosing interstitial lung diseases.The European respiratory journal · 2020Article
- Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme.Respiratory research · 2020Review
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14 authors at 5 institutions in 2 countries.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundFibrosing, non-idiopathic pulmonary fibrosis (non-IPF) interstitial lung diseases (fILDs) are a heterogeneous group of diseases characterized by a different amount of inflammation and fibrosis. Therapy is currently based on corticosteroids and/or immunomodulators. However, response to these therapies is highly variable, sometimes without meaningful improvement, especially in more fibrosing forms. Pirfenidone and nintedanib have recently demonstrated to reduce functional decline in patients with IPF. However, their antifibrotic mechanism makes these two drugs an interesting approach for treatment of fibrosing ILDs other than IPF.
objectivesWe here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients having a progressive phenotype during immunosuppressive therapy.
methodsPatients with a multidisciplinary team diagnosis of fibrosing non-IPF ILDs experiencing a progressive phenotype during treatment with corticosteroids and/or immunomodulators between October-2014 and January-2018 at our tertiary referral Center for ILDs were retrospectively analyzed. Antifibrotic therapy was administered after application with the respective health insurance company and after consent by the patient. Pulmonary-function-tests and follow-up visits were performed every 6 ± 1 months.
resultsEleven patients were treated with antifibrotic drugs (8 males, mean age 62 ± 12.8 years, mean FVC% 62.8 ± 22.3, mean DLCO% 35.5 ± 10.7, median follow-up under antifibrotic treatment 11.1 months). Patients had a diagnosis of unclassifiable ILD in 6 cases, pleuroparenchymal fibroelastosis in 2 cases, idiopathic-NSIP in 1 case, asbestos-related ILD in 1 case and Hermansky-Pudlak syndrome in 1 case. Treatment before antifibrotics consisted of corticosteroids in all patients: 5 combined with Azathioprin, 1 with either methotrexate or cyclophosphamide (i.v.). Ten patients were treated with pirfenidone (2403 mg/die) and 1 with nintedanib (300 mg/die). Median FVC was 56, 56, 50%, at time points - 24, - 12, - 6 before initiation, 44% at time of initiation and 46.5% at 6 months after initiation of antifibrotic treatment. Antifibrotic treatment was generally well tolerated with a need of dose reduction in 2 cases (rash and nausea) and early termination in 3 cases.
conclusionsAntifibrotic treatment may be a valuable treatment option in patients with progressive fibrosing non-IPF ILD if currently no other treatment options exist. However, prospective, randomized clinical trials are urgently needed to assess the real impact of antifibrotic therapy in these patients.
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