Faculty Opinions recommendation of Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature.

Author(s):  
Sergio Harari ◽  
Antonella Caminati
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.


2021 ◽  
Vol 10 (16) ◽  
pp. 3474
Author(s):  
Belén López-Muñiz Ballesteros ◽  
Marta López-Herranz ◽  
Ana Lopez-de-Andrés ◽  
Valentín Hernandez-Barrera ◽  
Rodrigo Jiménez-García ◽  
...  

(1) Background: To assess sex differences in the incidence, characteristics, procedures and outcomes of patients admitted with idiopathic pulmonary fibrosis (IPF); and to analyze variables associated with in-hospital mortality (IHM). (2) Methods: We analyzed data collected by the Spanish National Hospital Discharge Database, 2016–2019. (3) Results: We identified 13,278 hospital discharges (66.4% men) of IPF (primary diagnosis 32.33%; secondary diagnosis: 67.67%). Regardless of the diagnosis position, IPF incidence was higher among men than women, increasing with age. Men had 2.74 times higher IPF incidence than women. Comorbidity was higher for men in either primary or secondary diagnosis. After matching, men had higher prevalence of pulmonary embolism and pneumonia, and women of congestive heart failure, dementia, rheumatoid disease and pulmonary hypertension. Invasive ventilation, bronchoscopy and lung transplantation were received more often by men than women. IHM was higher among men with IPF as primary diagnosis than among women and increased with age in both sexes and among those who suffered cancer, pneumonia or required mechanical ventilation. (4) Conclusions: Incidence of IPF was higher among men than women, as well as comorbidity and use of bronchoscopy, ventilation and lung transplantation. IHM was worse among men than women with IPF as primary diagnosis, increasing with age, cancer, pneumonia or mechanical ventilation use.


2021 ◽  
Vol 28 (5) ◽  
pp. 987
Author(s):  
Mustafa Vayvada ◽  
Ayse Halis ◽  
Pinar Guney ◽  
Sevinc Citak ◽  
Ali Yiginsu ◽  
...  

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.


Author(s):  
Jonathan K. Alder ◽  
Rachel M. Sutton ◽  
Carlo J. Iasella ◽  
Mehdi Nouraie ◽  
Ritchie Koshy ◽  
...  

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