Idiopathic pulmonary fibrosis in the practice of a family doctor

Author(s):  
Vsevolod Vladimirovich Skvortsov ◽  
◽  
Dariya Nikolaevna Zadumina ◽  
Ekaterina Mikhailovna Skvortsova ◽  
Ekaterina Mikhailovna Tinaeva ◽  
...  

Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology, mainly characterized by a progressive deterioration in lung function due to active fibrosis. It is a variant of chronic progressive fibrosing interstitial pneumonia. The disease most often occurs between the ages of 50 and 70. According to the disease register in the Russian Federation, the prevalence of the disease in the Russian Federation is 4-7 people per hundred thousand of the population [1].Worldwide, the IPF incidence, prevalence and mortality increases over time [2, 3]. In a systematic review of the study by Hutchinson et al. [2] based on data on IPF morbidity and mortality in 21 countries between 1968 and 2012, it was determined that both morbidity and mortality have increased worldwide since 2000.

2018 ◽  
Vol 4 (4) ◽  
pp. 00156-2018 ◽  
Author(s):  
Kathryn M. Milne ◽  
Chrystal Chan ◽  
Jolene H. Fisher ◽  
Kaīssa de Boer ◽  
Christopher J. Ryerson

Narrative reviews are frequently accessed; however, the content and quality of review articles on idiopathic pulmonary fibrosis (IPF) have not been assessed.A systematic review assessed content and quality of narrative review articles that addressed the diagnosis or management of IPF and were published from 2001 to 2015. Article recommendations were assessed relative to contemporary IPF guidelines. Quality was assessed using the DISCERN instrument.Articles were predominantly written by physicians and published in respiratory journals. Conflicts of interest and sources of funding were reported in 52% and 24% of reviews, respectively. European authors were more likely to recommend bronchoscopy (adjusted p=0.02) and were more likely to recommend pirfenidone or nintedanib prior to publication of definitive clinical trials (adjusted p=0.04). A total of 39% of management-focused articles suggested therapies that were never recommended in guidelines. Predictors of higher article quality were citation of the contemporary IPF guideline (p=0.01) and more recent publication (p=0.001).Quality of reviews increased over time; however, review articles frequently made discordant recommendations compared to IPF guidelines. These findings indicate the need for authors, peer reviewers, editors and readers to critically appraise the content and quality of narrative reviews on IPF, and the need for frequent guideline updates to reflect new evidence.


2021 ◽  
Author(s):  
Tyler Pitre ◽  
Jasmine Mah ◽  
Sonya Cui ◽  
Melanie C. Zhang ◽  
Renata Husnudinov ◽  
...  

Abstract Background: Idiopathic pulmonary fibrosis (IPF) is a respiratory disorder of unknown etiology with a poor prognosis. There are novel therapies that have been studied in randomized controlled trials since the last network meta-analysis that may be of interest to academics and clinicians.Methods: We will perform a network meta-analysis on eligible randomized controlled trials of patients with IPF. We intend to search MEDLINE, EMBASE, Cochrane and clinicaltrials.org in order to complete a comprehensive search for adult IPF patients being treated with at least one of 21 of the selected medical therapies. A team will screen and extract eligible trials. We will perform Bayesian random-effects network meta-analysis. We will use GRADE and RoB 2.0 to assess the certainty and quality of the evidence. Discussion: There is a need for an updated meta-analysis on IPF medical therapies, including novel medical therapies. We intend on studying up to 21 medical therapies in the network meta-analysis to provide the most accurate and updated summary of the evidence for IPF treatments. Systematic review registrations: https://osf.io/afbhd/


Author(s):  
Elena Aloisio ◽  
Federica Braga ◽  
Chiara Puricelli ◽  
Mauro Panteghini

Abstract Objectives Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial disease with limited therapeutic options. The measurement of Krebs von den Lungen-6 (KL-6) glycoprotein has been proposed for evaluating the risk of IPF progression and predicting patient prognosis, but the robustness of available evidence is unclear. Methods We searched Medline and Embase databases for peer-reviewed literature from inception to April 2020. Original articles investigating KL-6 as prognostic marker for IPF were retrieved. Considered outcomes were the risk of developing acute exacerbation (AE) and patient survival. Meta-analysis of selected studies was conducted, and quantitative data were uniformed as odds ratio (OR) or hazard ratio (HR) estimates, with corresponding 95% confidence intervals (CI). Results Twenty-six studies were included in the systematic review and 14 were finally meta-analysed. For AE development, the pooled OR (seven studies) for KL-6 was 2.72 (CI 1.22–6.06; p=0.015). However, a high degree of heterogeneity (I2=85.6%) was found among selected studies. Using data from three studies reporting binary data, a pooled sensitivity of 72% (CI 60–82%) and a specificity of 60% (CI 52–68%) were found for KL-6 measurement in detecting insurgence of AE in IPF patients. Pooled HR (seven studies) for mortality prediction was 1.009 (CI 0.983–1.036; p=0.505). Conclusions Although our meta-analysis suggested that IPF patients with increased KL-6 concentrations had a significant increased risk of developing AE, the detection power of the evaluated biomarker is limited. Furthermore, no relationship between biomarker concentrations and mortality was found. Caution is also needed when extending obtained results to non-Asian populations.


Author(s):  
Conal Hayton ◽  
Dayle Terrington ◽  
Andrew M. Wilson ◽  
Nazia Chaudhuri ◽  
Colm Leonard ◽  
...  

BMJ Open ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. e050004
Author(s):  
Wenjuan Wu ◽  
Lingxiao Qiu ◽  
Jizhen Wu ◽  
Xueya Liu ◽  
Guojun Zhang

ObjectivesIdiopathic pulmonary fibrosis (IPF) has been defined as a distinctive type of chronic fibrotic disease, characterised by a progressive decline in lung function and a common histological pattern of interstitial pneumonia. To analyse the efficacy and safety of pirfenidone in the treatment of IPF, a systematic review and meta-analysis was performed.DesignThis is a meta-analysis study.ParticipantsPatients were diagnosed as IPF.InterventionsUse of pirfenidone.Primary and secondary outcomeProgression-free survival (PFS), acute exacerbation and worsening of IPF and Impact on adverse events.MeasuresThe inverse variance method for the random-effects model was used to summarise the dichotomous outcomes, risk ratios and 95% CIs.ResultsA total of 9 randomised controlled trials with 1011 participants receiving pirfenidone and 912 controls receiving placebo were summarised. The pooled result suggested a statistically significant difference inall-cause mortality after pirfenidone use, with a summarised relative ratio of 0.51 (p<0.01). Longer PFS was observed in patients receiving pirfenidone compared with those who were given placebo (p<0.01). The IPF groups presented a high incidence of adverse events with a pooled relative ratio of 3.89 (p<0.01).ConclusionsPirfenidone can provide survival benefit for patients with IPF. Pirfenidone treatment was also associated with a longer PFS, a lower incidence of acute exacerbation and worsening of IPF.


2021 ◽  
Vol 95 ◽  
Author(s):  
B. Bobić ◽  
V. Ćirković ◽  
I. Klun ◽  
T. Štajner ◽  
J. Srbljanović ◽  
...  

Abstract Taenia solium is a zoonotic parasite that causes taeniasis and cysticercosis in humans (as final hosts) and cysticercosis in pigs (as intermediate hosts). The Russian Federation (RF) is traditionally considered as endemic for this zoonosis. However, the epidemiological data on T. solium infection have not been reviewed for the past 20 years, in which time dynamic economical and societal changes have occurred in the RF. The aim of this systematic review was to analyse the status of T. solium infection in RF in the 2000–2019 period. A literature search was conducted, which collected published articles, grey literature and official data on the epidemiology of T. solium taeniasis and cysticercosis in the RF published from 2000. From a total of 2021 articles and 24 official reports originally returned by the search, data were extracted from 12 full text articles and 11 official reports. Taenia solium taeniasis was continuously reported in the RF between 2000 and 2019, with a tenfold decrease in the incidence, from 0.2 per 100,000 population in 2000 to 0.023/100,000 in 2019. Also, the number of administrative units where taeniasis was detected continuously decreased. Cysticercosis in pigs had a declining trend after 2006. In conclusion, although decreasing, T. solium infection is still endemic in several regions and suspected to be endemic in most of the RF.


2020 ◽  
pp. 106002802096445
Author(s):  
Enrica Di Martino ◽  
Alessio Provenzani ◽  
Patrizio Vitulo ◽  
Piera Polidori

Background: The comparative efficacy of pirfenidone, nintedanib, and pamrevlumab in slowing the rate of forced vital capacity (FVC) decline and mortality in patients with idiopathic pulmonary fibrosis (IPF) is unknown. Objective: To perform a systematic review and meta-analysis (MA) of these drugs for IPF. Methods: We searched CENTRAL, PubMed, EMBASE, ClincalTrials.gov, and the World Health Organization’s registry databases up to March 2020. Phase II/III randomized controlled trials in adults with IPF were eligible. The random-effect model was implemented calculating the effect size and respective 95% CI as Cohen’s d for change from baseline FVC (in percentage predicted and liters) and odds ratio (OR) for 10% reduction in FVC and all-cause mortality (ACM). Results: Six studies were included in the MA. For change from baseline in percentage predicted FVC, the MA indicated that the 3 drugs were more effective than placebo (pirfenidone: d=3.30%, 95% CI=2.15-4.45; nintedanib: d=3.15%, 95% CI=2.35-3.95; pamrevlumab: d=4.30%, 95% CI=0.45-8.15). These results are superimposable to those relating to change from baseline FVC in liters (pirfenidone: d=0.09L, 95% CI=0.04-0.14; nintedanib: d=0.13L, 95% CI=0.10-0.16; pamrevlumab: d=0.20L, 95% CI=0.05-0.35). Each drug had a positive effect on 10% reduction in FVC (pirfenidone: OR=0.57, 95% CI=0.45-0.74; nintedanib: OR=0.66, 95% CI=0.51-0.85; pamrevlumab: OR=0.24, 95% CI=0.08-0.73), but only pirfenidone showed an effect on ACM (OR=0.50; 95% CI=0.31-0.83). Conclusion and Relevance: This MA provided encouraging results on pamrevlumab efficacy in slowing the decline in FVC compared with pirfenidone and nintedanib. Actually, in phase 3, it could become a potential IPF treatment.


2019 ◽  
Vol 8 (1) ◽  
pp. 55-61 ◽  
Author(s):  
Aristeidis Skandamis ◽  
Chara Kani ◽  
Sophia L. Markantonis ◽  
Kyriakos Souliotis

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