Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease, characterised by progressive scarring of the lung and associated with a high burden of disease and early death. The pathophysiological understanding, clinical diagnostics and therapy of IPF have significantly evolved in recent years. While the recent introduction of the two antifibrotic drugs pirfenidone and nintedanib led to a significant reduction in lung function decline, there is still no cure for IPF; thus, new therapeutic approaches are needed. Currently, several clinical phase I-III trials are focusing on novel therapeutic targets. Furthermore, new approaches in nonpharmacological treatments in palliative care, pulmonary rehabilitation, lung transplantation, management of comorbidities and acute exacerbations aim to improve symptom control and quality of life. Here we summarise new therapeutic attempts and potential future approaches to treat this devastating disease.

/pdf/the-therapy-of-idiopathic-pulmonary-fibrosis-what-is-next-5cn9ro48xf.pdf

The therapy of idiopathic pulmonary fibrosis: what is next?

Objective. The aim of this study is to evaluate the efficacy and safety of pulmonary rehabilitation (PR) in patients with idiopathic pulmonary fibrosis (IPF). Methods. Embase, PubMed, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chongqing VIP (CQVIP), Wanfang Data, and Chinese Biomedical Literature Database (SinoMed) were comprehensively searched. Randomized controlled trials (RCTs) that investigated the effects of PR for IPF patients were included. Literature selection and data extraction were conducted by two review authors independently. The Cochrane Collaboration’s Risk of Bias tool and RevMan software (version 5.3) were used to evaluate the quality of studies and conduct statistical analysis, respectively. Results. Seven studies (190 participants) were included. PR had a significant effect on six-minute walk distance (6MWD) (MD:48.60; 95%CI: 29.03 to 68.18; Z=4.87, P<0.00001), and 6MWD was improved more in subgroup analysis including studies conducted in Asia (MD: 53.62; 95%CI: 30.48 to 76.66; Z=4.54, P<0.00001) and Europe (MD:54.10; 95% CI: 26.65 to 101.56; Z=2.23, P=0.03). Forced vital capacity (FVC%) was higher (MD: 3.69; 95%CI: 0.16 to 7.23; Z=2.05, P=0.04). St. George’s Respiratory Questionnaire (SGRQ)/IPF-specific SGRQ (SGRQ-I) total score was lower (MD: -7.87; 95% CI: -11.44 to -4.30; Z=4.32, P<0.0001). No significant effects were found for lung diffusing capacity determined by the single-breath technique (DLCO%) (MD: 3.02; 95%CI: -0.38 to 6.42; Z=1.74, P=0.08). Conclusions. This study suggests that PR may enhance exercise capacity and improve quality of life in IPF patients. Besides, PR may also delay the decline of lung function of patients with IPF. However, further research should more fully assess the efficacy and safety of PR for IPF.

/pdf/pulmonary-rehabilitation-for-exercise-tolerance-and-quality-45e2ovlx12.pdf

Pulmonary Rehabilitation for Exercise Tolerance and Quality of Life in IPF Patients: A Systematic Review and Meta-Analysis.

ABSTRACT Similarly to idiopathic pulmonary fibrosis (IPF), other interstitial lung diseases can develop progressive pulmonary fibrosis (PPF) characterized by declining lung function, a poor response to immunomodulatory therapies, and early mortality. The pathophysiology of disordered lung repair involves common downstream pathways that lead to pulmonary fibrosis in both IPF and PPF. The antifibrotic drugs, such as nintedanib, are indicated for the treatment of IPF and PPF, and new therapies are being evaluated in clinical trials. Clinical, radiographic, and molecular biomarkers are needed to identify patients with PPF and subgroups of patients likely to respond to specific therapies. This article reviews the evidence supporting the use of specific therapies in patients with IPF and PPF, discusses agents being considered in clinical trials, and considers potential biomarkers based on disease pathogenesis that might be used to provide a personalized approach to care.

/pdf/advances-in-the-management-of-idiopathic-pulmonary-fibrosis-2nmn1ftn.pdf

Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Background Few studies have examined the benefits of pulmonary rehabilitation in patients with fibrotic idiopathic pulmonary pneumonia (f-IIP). Here, we report the results of an observational study in routine clinical practice of home-based pulmonary rehabilitation for f-IIP patients. Methods A total of 112 consecutive patients (61 with idiopathic pulmonary fibrosis and 51 with fibrotic nonspecific interstitial pneumonitis) were enrolled, of whom 65 had mild-to-moderate disease (forced vital capacity (FVC) ≥50% predicted and diffusing capacity of the lung for carbon monoxide (DLCO) ≥30% predicted) and 47 had severe disease (FVC Results 6MST strokes, HADS Anxiety score and VSRQ score were each significantly improved at T2 (n=101), T8 (n=76) and T14 (n=62) compared with T0 values. The improvements in outcomes were not influenced by disease severity or subtype. Patients who completed the study had significantly better baseline FVC and DLCO values than those who did not. Conclusions Home-based pulmonary rehabilitation provides long-term benefits in exercise tolerance, anxiety and QoL for patients with f-IIP. Pulmonary rehabilitation should be prescribed systematically as part of the therapeutic arsenal for these patients.

https://openres.ersjournals.com/content/erjor/5/2/00045-2019.full.pdf

Long-term evaluation of home-based pulmonary rehabilitation in patients with fibrotic idiopathic interstitial pneumonias.

Introduction: This document presents the guidelines of the Polish Respiratory Society (PTChP, Polskie Towarzystwo Chorob Pluc) for diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), developed by a group of Polish experts. Material and methods: The recommendations were developed in the form of answers to previously formulated questions concer-ning everyday diagnostic and therapeutic challenges. They were developed based on a current literature review using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Results: We formulated 28 recommendations for diagnosis (8), pharmacological treatment (12) as well as non-pharma-cological and palliative therapy (8). The experts suggest that surgical lung biopsy (SLB) not be performed in patients with the probable usual interstitial pneumonia (UIP) pattern, with an appropriate clinical context and unanimous opinion of a  multidisciplinary team. The experts recommend using antifibrotic agents in IPF patients and suggest their use irrespective of the degree of functional impairment. As regards non-pharmacological and palliative treatment, strong re-commendations were formulated regarding pulmonary rehabilitation, oxygen therapy (in patients with chronic respiratory failure), preventive vaccinations as well as referring IPF patients to transplant centres. Table 1 presents an aggregate list of recommendations. Conclusions: The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.

/pdf/guidelines-of-the-polish-respiratory-society-for-diagnosis-3rv1kosypa.pdf

Guidelines of the Polish Respiratory Society for diagnosis and treatment of idiopathic pulmonary fibrosis.

Objective To investigate the short- and long-term effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis (IPF). Data sources An electronic search of MEDLINE, Embase and Cochrane Central databases along with hand search of relevant papers were performed on 15 March 2018. Review methods This study was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the literature for randomized controlled trials of pulmonary rehabilitation in IPF patients. The outcomes were exercise capacity and health-related quality of life. Two authors independently extracted data, assessed trial eligibility and risk of bias. Meta-analyses were performed using RevMan and STATA software. Results We extracted data from four randomized controlled trials (142 participants). At short-term follow-up, pulmonary rehabilitation significantly enhanced 6-minute walk distance (6-MWD; weighted mean difference (WMD) = 38.38, 95% confidence interval (CI) = 4.64-72.12, I2 = 60.7%; P Conclusion In patients with IPF, pulmonary rehabilitation showed short-term effects in enhancing exercise capacity and health-related quality of life, while it had no detectable effects at long-term follow-up.

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Short- and long-term effects of pulmonary rehabilitation for idiopathic pulmonary fibrosis: a systematic review and meta-analysis: