Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis

Fabrellas, Estrella Fernández; Sánchez, Ricardo Peris; Abad, Cristina Sabater; Samper, Gustavo Juan

doi:10.3390/medsci6020051

Cited by 48 publications

(34 citation statements)

References 57 publications

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“…IPF prevalence ranges from 1.25 to 23.4 cases per 100,000 population. It affects adults, especially men, aged over 50 years and has a poor prognosis, as death typically occurs at 2-4 years after diagnosis [5]. It is influenced by personal factors (smoking status, comorbidities, etc.).…”

Section: Introductionmentioning

confidence: 99%

Neutrophil-to-Lymphocyte Ratio and Systemic Immune-Inflammation Index—Biomarkers in Interstitial Lung Disease

et al. 2020

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Background and objectives: The aims of the study were to evaluate the utility of neutrophil-to-lymphocyte ratio (NLR) and the systemic immune-inflammation index (SII) as inflammation markers and prognostic factors in patients with known interstitial lung disease secondary to connective tissue diseases (CTD-ILD) compared with idiopathic pulmonary fibrosis (IPF). Materials and Methods: Forty-two patients with known interstitial lung disease (21 with IPF and 21 with CTD-ILD) and 42 control matched healthy patients were included. The NLR was calculated as the absolute neutrophil count divided by the absolute lymphocyte count, and the SII was calculated as follows: SII = platelets × neutrophils/lymphocytes, with the data being obtained from the patients data charts at admission, before any treatment. Results: our hypothesis was that in patients with interstitial lung disease NLR and SII would have higher values compared with patients with CTD-ILD or control healthy patients. The mean NLR value was 3.01 (±1.35) among patients with idiopathic pulmonary fibrosis, and 2.38 (±1.08) among patients with CTD-ILD without significant statistical difference (p = 0.92). There was however a clinically significant statistical difference when compared with the control group, where NLR was 2.00 (±1.05) (p = 0.003). SII values were 619.37 (±329.51) in patients with IPF, 671.55 (±365.73) in CTD-ILD group and 569.73 (±326.67) in healthy subjects (p = 0.13) Conclusions: A mean NLR value of 2.8 and a SII value over 500 in patients with connective diseases can become a marker of pulmonary interstitial involvement. In the context of non-exacerbated interstitial lung disease, NLR and SII have reduced numerical values, without being statistically correlated with prognosis when we compared with patients with connective tissue diseases without exacerbation or with healthy people, the cut off being of 2.4. However subsequent studies in larger patient samples might provide changes in these cut-off values.

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Section: Introductionmentioning

confidence: 99%

Neutrophil-to-Lymphocyte Ratio and Systemic Immune-Inflammation Index—Biomarkers in Interstitial Lung Disease

et al. 2020

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“…Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by extensive accumulation of abnormal extracellular matrix (ECM) in the lung and variable progression among patients, leading to death 3-5 years after diagnosis [1]. IPF is currently seen as the consequence of an unresolved wound healing [2], which is mediated by several pro-fibrotic factors and leads to an uncontrolled proliferation of myofibroblasts, responsible for the abnormal ECM production.…”

Section: Introductionmentioning

confidence: 99%

Pirfenidone anti-fibrotic effects are partially mediated by the inhibition of MUC1 bioactivation

2020

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Pirfenidone is a pleiotropic molecule approved to treat idiopathic pulmonary fibrosis (IPF). Pirfenidone has demonstrated to downregulate transforming growth factor-β1 (TGF-β1) cellular effects. However, its anti-fibrotic mechanism remains unclear. Here, we aim to analyze the effects of pirfenidone on the TGF-β1 canonical and non-canonical pathways, as well as, on the most characteristic IPF cellular processes. Results observed in this work showed that TGF-β1-induced canonical SMAD3 and noncanonical ERK1/2 phosphorylations were not inhibited by pirfenidone in alveolar A549 and lung fibroblasts MRC5 cells. In contrast, pirfenidone inhibited TGF-β1-induced MUC1-CT Thr 41 (1224) and Tyr 46 (1229) phosphorylations, thus reducing the β-catenin activation. Additionally, immunoprecipitation and immunofluorescence studies in ATII cells and lung fibroblasts showed that pirfenidone inhibited the formation and nuclear translocation of the transcriptional fibrotic TGF-β1-induced phospho-SMAD3/MUC1-CT/active-β-catenin complex, and consequently the SMAD-binding element activation (SBE). This study provided also evidence of the inhibitory effect of pirfenidone on the TGF-β1-induced ATII to mesenchymal and fibroblast to myofibroblast transitions, fibroblast proliferation and ATII and fibroblast senescence. Therefore, it indicates that pirfenidone's inhibitory effect on TGF-β1-induced fibrotic cellular processes is mediated by the inhibition of MUC1-CT phosphorylation, β-catenin activation, nuclear complex formation of phospho-SMAD3/MUC1-CT/active β-catenin and SBE activation, which may be of value to further develop anti-fibrotic IPF therapies.

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“…The early identification of these complications is crucial, in order to increase the survival of the disease; in addition, the results of recent genetic studies, in combination with clinical and radiological features, are trying to introduce some biomarkers for monitoring disease [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

et al. 2020

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Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications—which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management.

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Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis

Cited by 48 publications

References 57 publications

Neutrophil-to-Lymphocyte Ratio and Systemic Immune-Inflammation Index—Biomarkers in Interstitial Lung Disease

Neutrophil-to-Lymphocyte Ratio and Systemic Immune-Inflammation Index—Biomarkers in Interstitial Lung Disease

Pirfenidone anti-fibrotic effects are partially mediated by the inhibition of MUC1 bioactivation

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

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