Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases
Abstract Background Fibrosing, 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, respon...
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2019-11-01
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Series: | BMC Pulmonary Medicine |
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Online Access: | http://link.springer.com/article/10.1186/s12890-019-0937-0 |
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record_format |
Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sebastiano Emanuele Torrisi Nicolas Kahn Julia Wälscher Nilab Sarmand Markus Polke Kehler Lars Monika Eichinger Claus Peter Heussel Stefano Palmucci Francesca Maria Sambataro Gianluca Sambataro Domenico Sambataro Carlo Vancheri Michael Kreuter |
spellingShingle |
Sebastiano Emanuele Torrisi Nicolas Kahn Julia Wälscher Nilab Sarmand Markus Polke Kehler Lars Monika Eichinger Claus Peter Heussel Stefano Palmucci Francesca Maria Sambataro Gianluca Sambataro Domenico Sambataro Carlo Vancheri Michael Kreuter Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases BMC Pulmonary Medicine Interstitial lung disease Nintedanib Pirfenidone Progressive fibrosing interstitial lung diseases Real-world experience IPAF |
author_facet |
Sebastiano Emanuele Torrisi Nicolas Kahn Julia Wälscher Nilab Sarmand Markus Polke Kehler Lars Monika Eichinger Claus Peter Heussel Stefano Palmucci Francesca Maria Sambataro Gianluca Sambataro Domenico Sambataro Carlo Vancheri Michael Kreuter |
author_sort |
Sebastiano Emanuele Torrisi |
title |
Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases |
title_short |
Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases |
title_full |
Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases |
title_fullStr |
Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases |
title_full_unstemmed |
Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases |
title_sort |
possible value of antifibrotic drugs in patients with progressive fibrosing non-ipf interstitial lung diseases |
publisher |
BMC |
series |
BMC Pulmonary Medicine |
issn |
1471-2466 |
publishDate |
2019-11-01 |
description |
Abstract Background Fibrosing, 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. Objectives We here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients having a progressive phenotype during immunosuppressive therapy. Methods Patients 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. Results Eleven 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. Conclusions Antifibrotic 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. |
topic |
Interstitial lung disease Nintedanib Pirfenidone Progressive fibrosing interstitial lung diseases Real-world experience IPAF |
url |
http://link.springer.com/article/10.1186/s12890-019-0937-0 |
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doaj-31ba4f3e3ed3410c893583025e50db572020-11-25T04:07:18ZengBMCBMC Pulmonary Medicine1471-24662019-11-011911910.1186/s12890-019-0937-0Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseasesSebastiano Emanuele Torrisi0Nicolas Kahn1Julia Wälscher2Nilab Sarmand3Markus Polke4Kehler Lars5Monika Eichinger6Claus Peter Heussel7Stefano Palmucci8Francesca Maria Sambataro9Gianluca Sambataro10Domenico Sambataro11Carlo Vancheri12Michael Kreuter13Center for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchCenter for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchCenter for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchCenter for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchCenter for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchCenter for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchDepartment of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik, University of Heidelberg and Translational Lung Research Center Heidelberg, member of the German Center for Lung ResearchDepartment of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik, University of Heidelberg and Translational Lung Research Center Heidelberg, member of the German Center for Lung ResearchRadiology I Unit, Department of Medical Surgical Sciences and Advanced Technologies, University Hospital “Policlinico-Vittorio Emanuele”Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik, University of Heidelberg and Translational Lung Research Center Heidelberg, member of the German Center for Lung ResearchDepartment of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of CataniaDepartment of Clinical and Experimental Medicine, Internal Medicine Unit, Cannizzaro Hospital, University of CataniaDepartment of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of CataniaCenter for interstitial and rare lung diseases, Pneumology, Thoraxklinik, University of Heidelberg, Germany and German Center for Lung ResearchAbstract Background Fibrosing, 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. Objectives We here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients having a progressive phenotype during immunosuppressive therapy. Methods Patients 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. Results Eleven 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. Conclusions Antifibrotic 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.http://link.springer.com/article/10.1186/s12890-019-0937-0Interstitial lung diseaseNintedanibPirfenidoneProgressive fibrosing interstitial lung diseasesReal-world experienceIPAF |