Cause of mortality and sarcopenia in patients with idiopathic pulmonary fibrosis receiving antifibrotic therapy

Respirology ◽  
2020 ◽  
Author(s):  
Yuzo Suzuki ◽  
Yuya Aono ◽  
Masato Kono ◽  
Hirotsugu Hasegawa ◽  
Koushi Yokomura ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Antifibrotic Therapy

scholarly journals Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

2021 ◽  
Vol 10 (11) ◽  
pp. 2285
Author(s):  
John N. Shumar ◽  
Abhimanyu Chandel ◽  
Christopher S. King
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Treatment Pathway ◽  
Antifibrotic Therapy ◽  
New Treatment ◽  
Fibrotic Lung Diseases

Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

scholarly journals Switching antifibrotics in patients with idiopathic pulmonary fibrosis: a multi-center retrospective cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuzo Suzuki ◽  
Kazutaka Mori ◽  
Yuya Aono ◽  
Masato Kono ◽  
Hirotsugu Hasegawa ◽  
...  
Keyword(s):  
Cohort Study ◽  
Propensity Score ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Survival Times ◽  
Antifibrotic Therapy ◽  
Median Period

Abstract Background Currently, there are two antifibrotics used to treat idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. Antifibrotics slow disease progression by reducing the annual decline of forced vital capacity (FVC), which possibly improves outcomes in IPF patients. During treatment, patients occasionally switch antifibrotic treatments. However, prognostic implication of changing antifibrotics has not yet been evaluated. Methods This multi-center retrospective cohort study examined 262 consecutive IPF patients who received antifibrotic therapy. Antifibrotic agents were switched in 37 patients (14.1%). The prognoses were compared between the patient cohort that switched antifibrotics (Switch-IPF) and those without (Non-Switch-IPF) using propensity-score matched analyses. Results The median period between the initiation of antifibrotic therapy and the drug switch was 25.8 (12.7–35.3) months. The most common reasons for the switch were disease progression (n = 17) followed by gastrointestinal disorders (n = 12). Of the 37 patients that switched antifibrotics, only eight patients disrupted switched antifibrotics by their adverse reactions. The overall prognosis of the Switch-IPF cohort was significantly better than the Non-Switch-IPF cohort (median periods: 67.2 vs. 27.1 months, p < 0.0001). In propensity-score matched analyses that were adjusted to age, sex, FVC (%), history of acute exacerbation, and usage of long-term oxygen therapy, the Switch-IPF cohort had significantly longer survival times than the Non-Switch-IPF group (median 67.2 vs. 41.3 months, p = 0.0219). The second-line antifibrotic therapy showed similar survival probabilities than those in first-line antifibrotic therapy in multistate model analyses. Conclusion Switching antifibrotics is feasible and may improve prognosis in patients with IPF. A further prospective study will be required to confirm clinical implication of switching the antifibrotics.

scholarly journals Physician characteristics associated with treatment initiation patterns in idiopathic pulmonary fibrosis

2019 ◽  
Vol 16 ◽  
pp. 147997311987967 ◽  
Author(s):  
Peter P LaCamera ◽  
Susan L Limb ◽  
Tmirah Haselkorn ◽  
Elizabeth A Morgenthien ◽  
John L Stauffer ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Treatment Initiation ◽  
Group Versus ◽  
Direct Patient Care ◽  
Real World Data ◽  
Antifibrotic Therapy ◽  
Therapy Initiation ◽  
Physician Characteristics ◽  
Delayed Group

Pirfenidone and nintedanib are oral antifibrotic agents approved for the treatment of idiopathic pulmonary fibrosis (IPF). Real-world data on factors that influence IPF treatment decisions are limited. Physician characteristics associated with antifibrotic therapy initiation following an IPF diagnosis were examined in a sample of US pulmonologists. An online, self-administered survey was fielded to pulmonologists between April 10, 2017, and May 17, 2017. Pulmonologists were included if they spent >20% of their time in direct patient care and had ≥5 patients with IPF receiving antifibrotics. Participants answered questions regarding timing and reasons for considering the initiation of antifibrotic therapy after an IPF diagnosis. A total of 169 pulmonologists participated. The majority (81.7%) considered initiating antifibrotic therapy immediately after IPF diagnosis all or most of the time (immediate group), while 18.3% considered it only some of the time or not at all (delayed group). Pulmonologists in the immediate group were more likely to work in private practice (26.1%), have a greater mean percentage of patients receiving antifibrotic therapy (60.8%), and decide to initiate treatment themselves (31.2%) versus those in the delayed group (16.1%, 30.5%, and 16.1%, respectively). Most pulmonologists consider initiating antifibrotic treatment immediately after establishing an IPF diagnosis all or most of the time versus using a “watch-and-wait” approach. Distinguishing characteristics between pulmonologists in the immediate group versus the delayed group included practice setting, percentage of patients receiving antifibrotic therapy, and the decision-making dynamics between the patient and the pulmonologist.

scholarly journals Differential diagnosis of idiopathic pulmonary fibrosis

2020 ◽  
Vol 92 (3) ◽  
pp. 102-108
Author(s):  
E. I. Shmelev ◽  
A. E. Ergeshov ◽  
V. Ya. Gergert
Keyword(s):  
Differential Diagnosis ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Respiratory Diseases ◽  
Clinical Signs ◽  
Interstitial Lung Diseases ◽  
Antifibrotic Therapy ◽  
Inflammatory Drugs ◽  
Diffuse Lung

The review is devoted to the urgent problem of modern pulmonology: the differential diagnosis of idiopathic pulmonary fibrosis (ILF). ILF occupies a special place among many interstitial lung diseases for a number of reasons: 1) it is a deadly disease; 2) early diagnosis and adequate antifibrotic therapy significantly extend the life expectancy of patients; 3) anti-inflammatory drugs (corticosteroids) and cytostatics with ILF that are widely used in other forms of interstitial lung diseases are ineffective and accelerate the progression of the process; 4) the commonality of the main clinical signs (increasing respiratory failure) of various interstitial lung diseases. The list of respiratory diseases with which ILF should be differentiated is huge, and if with diffuse lung lesions of a known nature (disseminated pulmonary tuberculosis, pneumoconiosis, etc.) with a certain experience/qualification, the diagnosis is relatively simple, then the isolation of ILF from the group of idiopathic interstitial pneumonias always represents certain difficulties. The main methods used in the diagnosis of ILF are summarized taking into account current international and national recommendations.

scholarly journals A clinical case of idiopathic pulmonary fibrosis against the background of comorbid pathology

2021 ◽  
Vol 20 (3) ◽  
pp. 225-232
Author(s):  
A. V. Teteneva ◽  
V. V. Kalyuzhin ◽  
G. M. Chernyavskaya ◽  
I. D. Bespalova ◽  
G. E. Chernogoryuk ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Case ◽  
Clinical Laboratory ◽  
Diagnostic Algorithm ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Antifibrotic Therapy ◽  
The Past ◽  
Tomsk Region

Idiopathic pulmonary fibrosis (IPF) is one of the most common diseases in the group of interstitial lung diseases, which is characterized by persistent progression and poor prognosis. Over the past decade, experts have made significant progress in developing a diagnostic algorithm for IPF patients. This algorithm includes analysis of clinical, laboratory, and instrumental data, primarily the results of high-resolution computed tomography (HRCT). Precise adherence to the diagnostic algorithm and correct interpretation of HRCT data are prerequisites for IPF diagnosis.Specialists of the Tomsk region have developed routing of patients with suspected IPF. The presented clinical case is a successful example of adhering to this algorithm. Wide implementation of modern diagnostic algorithms into diagnosis and treatment of IPF and quality improvement of imaging methods, primarily HRCT, carried out as a part of the differential diagnosis, open up prospects for early diagnosis of this pathology. A timely prescribed antifibrotic therapy (nintedanib, pirfenidone) in IPF allows to slow down pathological progression and improves the prognosis.

Chronic fibrosing progressing interstitial lung disease: a decision of Multidisciplinary Expert Board

2021 ◽  
Vol 31 (4) ◽  
pp. 505-510
Author(s):  
S. N. Avdeev ◽  
S. Yu. Chikina ◽  
I. E. Tyurin ◽  
A. S. Belevskiy ◽  
S. A. Terpigorev ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Russian Federation ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Diagnosis And Treatment ◽  
Antifibrotic Therapy

Introduction. The natural course of some interstitial lung diseases (ILD) is characterized by progressive fibrosing phenotype resembling idiopathic pulmonary fibrosis (IPF). Until recently, the antifibrotic drug nintedanib was approved for treatment of the only fibrosing ILD which was IPF. A new indication for this drug which has been registered in Russian Federation in 2021 includes other fibrosing ILDs with progressive phenotype (PF-ILDs) and ILD associated with systemic scleroderma (SS-ILD).The aim of this publication is to describe general considerations of the decision of Multidisciplinary Expert Board on diagnosis and treatment of PF-ILDs including SS-ILD.Results. According to the extension in nintedanib use mentioned above, the Expert Board created an algorithm for diagnosis and treatment of patients with PF-ILDs and criteria for nuntedanib administration in PF-ILDs.Conclusion. Antifibrotic therapy is needed for patients with PF-ILDs with the failure of the stanrard therapy. In those patients antifibrotic treatment should be initiated as early as possible to better preserve the lung function.

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