scholarly journals Acute exacerbations of progressive-fibrosing interstitial lung diseases

2018 ◽  
Vol 27 (150) ◽  
pp. 180071 ◽  
Author(s):  
Martin Kolb ◽  
Benjamin Bondue ◽  
Alberto Pesci ◽  
Yasunari Miyazaki ◽  
Jin Woo Song ◽  
...  
Keyword(s):  
Acute Exacerbation ◽  
Lung Diseases ◽  
Current Knowledge ◽  
Management Strategies ◽  
Interstitial Lung Diseases ◽  
Computerised Tomography ◽  
Acute Exacerbations ◽  
Clinically Significant ◽  
Randomised Controlled ◽  
Antifibrotic Drugs

Acute exacerbation of interstitial lung disease (ILD) is associated with a poor prognosis and high mortality. Numerous studies have documented acute exacerbation in idiopathic pulmonary fibrosis (IPF), but less is known about these events in other ILDs that may present a progressive-fibrosing phenotype. We propose defining acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs. Drawing on observations in IPF, it is suspected that epithelial injury or proliferation and autoimmunity are risk factors for acute exacerbation in ILDs that may present a progressive-fibrosing phenotype, but further studies are required. Current acute exacerbation management strategies are based on recommendations in IPF, but no randomised controlled trials of acute exacerbation management have been performed. Although there are no formal strategies to prevent the development of acute exacerbation, possible approaches include antifibrotic drugs (such as nintedanib and pirfenidone), and minimising exposure to infection, airborne irritants and pollutants. This review discusses the current knowledge of acute exacerbation of ILDs that may present a progressive-fibrosing phenotype and acknowledges limitations of the data available.

scholarly journals Clinical significance of respiratory virus detection in patients with acute exacerbation of interstitial lung diseases

2018 ◽  
Vol 136 ◽  
pp. 88-92 ◽  
Author(s):  
Takeshi Saraya ◽  
Hirokazu Kimura ◽  
Daisuke Kurai ◽  
Masaki Tamura ◽  
Yukari Ogawa ◽  
...  
Keyword(s):  
Acute Exacerbation ◽  
Clinical Significance ◽  
Lung Diseases ◽  
Respiratory Virus ◽  
Virus Detection ◽  
Interstitial Lung Diseases

scholarly journals Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

2018 ◽  
Vol 27 (150) ◽  
pp. 180074 ◽  
Author(s):  
Luca Richeldi ◽  
Francesco Varone ◽  
Miguel Bergna ◽  
Joao de Andrade ◽  
Jeremy Falk ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Occupational Exposures ◽  
Interstitial Lung Diseases ◽  
Therapeutic Options ◽  
Current Evidence ◽  
Pharmacological Management ◽  
The Status ◽  
Nonspecific Interstitial Pneumonia ◽  
Randomised Controlled

A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to patients with these diseases. However, this treatment regimen may not be effective, adequate on its own or well tolerated, suggesting that there is a pressing need for efficacious and better tolerated therapies. Currently, the only approved treatments to slow disease progression in patients with IPF are nintedanib and pirfenidone. Similarities in pathobiological mechanisms leading to fibrosis between IPF and other ILDs that may present a progressive-fibrosing phenotype provide a rationale to suggest that nintedanib and pirfenidone may be therapeutic options for patients with the latter diseases.This review provides an overview of the therapeutic options currently available for patients with fibrosing ILDs, including fibrosing ILDs that may present a progressive phenotype, and explores the status of the randomised controlled trials that are underway to determine the efficacy and safety of nintedanib and pirfenidone.

scholarly journals Progressive Fibrosing Interstitial Lung Diseases: A Current Perspective

Biomedicines ◽  
2021 ◽  
Vol 9 (9) ◽  
pp. 1237
Author(s):  
Carlo Albera ◽  
Giulia Verri ◽  
Federico Sciarrone ◽  
Elena Sitia ◽  
Mauro Mangiapia ◽  
...  
Keyword(s):  
Lung Fibrosis ◽  
Lung Diseases ◽  
Current Knowledge ◽  
Relevant Literature ◽  
Interstitial Lung Diseases ◽  
Current Perspective ◽  
Similar Disease ◽  
Diagnostic Approaches ◽  
Disease Behavior ◽  
A Current

Interstitial lung diseases (ILDs) are a large and diverse group of rare and chronic respiratory disorders, with idiopathic pulmonary fibrosis (IPF) being the most common and best-studied member. Increasing interest in fibrosis as a therapeutic target and the appreciation that fibrotic mechanisms may be a treatable target of IPF prompted the development and subsequent approval of the antifibrotics, pirfenidone and nintedanib. The management of ILDs has changed considerably following an understanding that IPF and some ILDs share similar disease behavior of progressive fibrosis, termed “progressive fibrosing phenotype”. Indeed, antifibrotic treatment has shown to be beneficial in ILDs characterized by the progressive fibrosing phenotype. This narrative review summarizes current knowledge in the field of progressive fibrosing ILDs. Here, we discuss the clinical characteristics and pathogenesis of lung fibrosis and highlight relevant literature concerning the mechanisms underlying progressive fibrosing ILDs. We also summarize current diagnostic approaches and the available treatments of progressive fibrosing ILDs and address the optimization of treating progressive fibrosing ILDs with antifibrotics in clinical practice.

scholarly journals Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Sebastiano Emanuele Torrisi ◽  
Nicolas Kahn ◽  
Julia Wälscher ◽  
Nilab Sarmand ◽  
Markus Polke ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Randomized Clinical Trials ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Insurance Company ◽  
Meaningful Improvement ◽  
Tertiary Referral Center ◽  
Antifibrotic Therapy ◽  
Antifibrotic Drugs

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.

Acute exacerbation admissions of fibrosing interstitial lung diseases – 3 years study

2019 ◽  
Author(s):  
Sónia Isabel Silva Guerra ◽  
Mariana Conceição ◽  
Ângela Cunha ◽  
Joana Correia ◽  
Jorge Vale ◽  
...  
Keyword(s):  
Acute Exacerbation ◽  
Lung Diseases ◽  
Interstitial Lung Diseases

Recurrent Acute Exacerbations of Fibrotic Interstitial Lung Diseases

2019 ◽  
Author(s):  
A. Suzuki ◽  
Y. Kondoh ◽  
K.K. Brown ◽  
T. Kimura ◽  
K. Kataoka ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Acute Exacerbations

scholarly journals Three cases of sequential treatment with nintedanib following pulsed-dose corticosteroids for acute exacerbation of interstitial lung diseases

2021 ◽  
Vol 33 ◽  
pp. 101385
Author(s):  
Kazuki Nakashima ◽  
Toyoshi Yanagihara ◽  
Sae Ishida ◽  
Naruhiko Ogo ◽  
Ayaka Egashira ◽  
...  
Keyword(s):  
Acute Exacerbation ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Sequential Treatment

scholarly journals Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be

2021 ◽  
Vol 10 (6) ◽  
pp. 1330
Author(s):  
Tinne Goos ◽  
Laurens J. De Sadeleer ◽  
Jonas Yserbyt ◽  
Geert M. Verleden ◽  
Marie Vermant ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Immunosuppressive Agents ◽  
Significant Proportion ◽  
Standard Of Care ◽  
Interstitial Lung Diseases ◽  
Pharmacological Management ◽  
Shared Genetic ◽  
Antifibrotic Drugs

A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway—self-sustaining fibroproliferation—support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. While antifibrotic drugs became the standard of care in IPF, immunosuppressive agents are still the mainstay of treatment in non-IPF fibrosing ILD (F-ILD). However, recently, randomized placebo-controlled trials have demonstrated the efficacy and safety of antifibrotic treatment in systemic sclerosis-associated F-ILD and a broad range of F-ILDs with a progressive phenotype. This review summarizes the current pharmacological management and highlights the unmet needs in patients with non-IPF ILD.

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