scholarly journals Telomeric repeat-containing ribonucleic acid (TERRA) expression in patients with idiopathic pulmonary fibrosis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Manal M. El-Desoky ◽  
Asem A. Hewidy ◽  
Ahmed M. Fouda ◽  
Fatma Azzahraa Hisham
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Telomeric Repeat ◽  
Expression Level ◽  
Strongly Correlated ◽  
High Resolution Computed Tomography ◽  
Non Invasive ◽  
Non Coding Rna ◽  
Extent Score ◽  
Long Non Coding Rna

Abstract Background Idiopathic pulmonary fibrosis (IPF) represents a chronic disease with a progressive course. It is characterized by excessive lung scarring that ultimately contributes to irreversible lung function reduction. Interestingly, a type of long non-coding RNA termed as telomeric repeat-containing RNA (TERRA) is linked to fibrosis pathophysiology, including IPF. In this study, the expression profile of TERRA was investigated in IPF patients on radiological diagnosis [unusual interstitial pattern (UIP) in high-resolution computed tomography (HRCT)] to evaluate whether it could be employed as a reliable diagnostic biomarker. Results TERRA expression level was significantly higher in IPF patients over healthy controls. The expression level was significantly inversely correlated with the percentage of forced vital capacity predicted (FVC% predicted). By contrast, it was significantly directly correlated with HRCT reticular extent score. Conclusion TERRA expression is an essential biomarker in peripheral blood of IPF patients, providing a valuable non-invasive tool for IPF diagnosis. Moreover, TERRA expression is strongly correlated with UIP in HRCT reticular extent score.

scholarly journals Targeting the DNM3OS / miR-199a~214 cluster for the treatment of fibroproliferative diseases

2018 ◽  
Author(s):  
G. Savary ◽  
M. Buscot ◽  
E. Dewaeles ◽  
S. Diazzi ◽  
N. Nottet ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Mature Mirnas ◽  
Kidney Fibrosis ◽  
Downstream Effector ◽  
Non Coding Rna ◽  
Fibrotic Diseases ◽  
Fibrotic Disorders ◽  
Long Non Coding Rna ◽  
Lung And Kidney

AbstractGiven the paucity of effective treatments for fibrotic disorders, new insights into the deleterious mechanisms controlling fibroblast activation, the key cell type driving the fibrogenic process, are essential to develop new therapeutic strategies. Here, we identified the long non-coding RNA DNM3OS as a critical downstream effector of TGF-β-induced myofibroblast activation. Mechanistically, DNM3OS regulates this process in trans by giving rise to 3 distinct profibrotic mature miRNAs (i.e. miR-199a-5p/3p and miR-214-3p), which influence both SMAD and non-SMAD components of TGF-β signaling in a multifaceted way, through two modes of action consisting of either signal amplification or mediation. Finally, we provide preclinical evidence that interfering with DNM3OS function using distinct strategies not only prevents lung and kidney fibrosis but also improves established lung fibrosis, providing thus a novel paradigm for the treatment of refractory fibrotic diseases such as idiopathic pulmonary fibrosis.One Sentence SummaryThe DNM3OS lncRNA is a reservoir of fibromiRs with major functions in fibroblast response to TGF-β and represents a valuable therapeutic target for refractory fibrotic diseases such as idiopathic pulmonary fibrosis (IPF).

scholarly journals Chemotherapy in idiopathic pulmonary fibrosis and small-cell lung cancer with poor lung function

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiyue Zhang ◽  
Wei Li ◽  
Chunyan Li ◽  
Jie Zhang ◽  
Zhenzhong Su
Keyword(s):  
Lung Cancer ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Small Cell Lung Cancer ◽  
Cell Lung Cancer ◽  
Small Cell ◽  
Small Cell Lung ◽  
High Resolution Computed Tomography ◽  
Chemotherapeutic Regimen

Abstract Background Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with unclear pathogenesis. IPF is considered as a risk factor for lung cancer. Compared to other lung cancers, small-cell lung cancer (SCLC) has a lower incidence, but has a more aggressive course. Patients with IPF and SCLC have a lower survival rate, more difficult treatment, and poorer prognosis. Case presentation Case 1 was of a 66-year-old man with IPF for 5 years, who was admitted to our hospital for dyspnea. Case 2 was of a 68-year-old woman, who presented with chest pains, cough, and dyspnea. Both patients had extremely poor lung function. High-resolution computed tomography and pathology revealed that both patients had IPF and SCLC. Chemotherapy comprising nedaplatin (80 mg/m2) and etoposide (100 mg for 5 days) was initiated for both patients. Antifibrotic agents were continued during the chemotherapeutic regimen. Both patients showed improvement in their condition after treatment. Conclusion The favorable outcomes in these 2 cases suggests that chemotherapy is worth considering in the management of patients having SCLC and IPF with poor lung function.

scholarly journals S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis

Respiration ◽  
2021 ◽  
pp. 1-34
Author(s):  
Jürgen Behr ◽  
Andreas Günther ◽  
Francesco Bonella ◽  
Julien Dinkel ◽  
Ludger Fink ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Diagnosis ◽  
Diagnostic Process ◽  
High Resolution Computed Tomography ◽  
Cellular Analysis ◽  
Golden Standard ◽  
Technical Advances ◽  
Transbronchial Lung Cryobiopsy ◽  
Effective Diagnosis

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic process mandatory. The goal of this guideline is to foster early, confident, and effective diagnosis of IPF. The guideline focusses on the typical clinical context of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardized questionnaires, serologic testing, and cellular analysis of bronchoalveolar lavage. High-resolution computed tomography remains crucial in the diagnostic workup. If it is necessary to obtain specimens for histology, transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom a bronchoscopic diagnosis did not provide the information needed. After all, IPF is a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.

Patients With Idiopathic Pulmonary Fibrosis Admitted to the ICU With Acute Respiratory Failure—A Reevaluation of the Risk Factors and Outcomes

2021 ◽  
pp. 088506662198924
Author(s):  
Matthew Schrader ◽  
Matheni Sathananthan ◽  
Niranjan Jeganathan
Keyword(s):  
Risk Factors ◽  
Respiratory Failure ◽  
Mortality Rate ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Respiratory Failure ◽  
Small Sample ◽  
Apache Score ◽  
Non Invasive ◽  
Organ Failures

Introduction: Idiopathic pulmonary fibrosis (IPF) patients admitted to the ICU with acute respiratory failure (ARF) are known to have a poor prognosis. However, the majority of the studies published to date are older and had small sample sizes. Given the advances in ICU care since the publication of these studies, we sought to reevaluate the outcomes and risk factors associated with mortality in these patients. Methods: Retrospective study using a large multi-center ICU database. We identified 411 unique patients with IPF admitted with ARF between 2014-2015. Results: Of all IPF patients admitted to the ICU with ARF, 81.3% required mechanical ventilation (MV): 48.9% invasive and 32.4% non-invasive alone. The hospital mortality rate was 34.5% for all patients; 48.8% in patients requiring invasive MV, 21.8% in those requiring non-invasive MV and 19.5% with no MV. In multiple regression analyses, age, APACHE score, invasive MV, and hyponatremia at admission were associated with increased mortality whereas post-op status was associated with lower mortality. In patients requiring invasive MV, baseline PaO2/FiO2 ratio was also predictive of mortality. Non-pulmonary organ failures were present in less than 20% of the patients. Conclusions: Although the overall mortality rate for IPF patients admitted to the ICU with ARF has improved, the mortality rates for patients requiring invasive MV remains high at approximately 50%. Older age, high APACHE score, and low baseline PaO2/FiO2 ratio are factors predictive of increased mortality in this population.

scholarly journals Diagnostic Disparity of Previous and Revised American Thoracic Society Guidelines for Idiopathic Pulmonary Fibrosis

2015 ◽  
Vol 22 (2) ◽  
pp. 86-90 ◽  
Author(s):  
Lee Fidler ◽  
Shane Shapera ◽  
Shikha Mittoo ◽  
Theodore K Marras
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Diagnostic Criteria ◽  
Retrospective Chart Review ◽  
Patient Characteristics ◽  
Disease Diagnosis ◽  
American Thoracic Society ◽  
Patient Classification ◽  
High Resolution Computed Tomography ◽  
The Impact

BACKGROUND: A revised guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) was formulated by the American Thoracic Society (ATS) in 2011 to improve disease diagnosis and provide a simplified algorithm for clinicians. The impact of these revisions on patient classification, however, remain unclear.OBJECTIVE: To examine the concordance between diagnostic guidelines to understand how revisions impact patient classification.METHODS: A cohort of 54 patients with either suspected IPF or a working diagnosis of IPF was evaluated in a retrospective chart review, in which patient data were examined according to previous and revised ATS guidelines. Patient characteristics influencing the fulfillment of diagnostic criteria were compared using one-way ANOVA and χ2tests.RESULTS: Revised and previous guideline criteria for IPF were met in 78% and 83% of patients, respectively. Revised guidelines modified a classification based on previous guidelines in 28% of cases. Fifteen percent of patients meeting previous ATS guidelines failed to meet revised criteria due to a lack of honeycombing on high-resolution computed tomography and the absence of a surgical lung biopsy. Patients failing to meet previous and revised diagnostic criteria for IPF were younger.CONCLUSION: The revised guidelines for the diagnosis of IPF classify a substantial proportion of patients differently than the previous guidelines.

scholarly journals Effects of Nintedanib on Quantitative Lung Fibrosis Score in Idiopathic Pulmonary Fibrosis

2020 ◽  
Vol 14 (1) ◽  
pp. 22-31
Author(s):  
Lisa Lancaster ◽  
Jonathan Goldin ◽  
Matthias Trampisch ◽  
Grace Hyun Kim ◽  
Jonathan Ilowite ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Lung Fibrosis ◽  
Open Label ◽  
High Resolution Computed Tomography ◽  
Double Blind ◽  
Phase Iiib ◽  
Exploratory Data ◽  
Relative Change

Background: Nintedanib slows disease progression in patients with Idiopathic Pulmonary Fibrosis (IPF) by reducing decline in Forced Vital Capacity (FVC). The effects of nintedanib on abnormalities on high-resolution computed tomography scans have not been previously studied. Objective: We conducted a Phase IIIb trial to assess the effects of nintedanib on changes in Quantitative Lung Fibrosis (QLF) score and other measures of disease progression in patients with IPF. Methods: 113 patients were randomized 1:1 to receive nintedanib 150 mg bid or placebo double-blind for ≥6 months, followed by open-label nintedanib. The primary endpoint was the relative change from baseline in QLF score (%) at month 6. Analyses were descriptive and exploratory. Results: Adjusted mean relative changes from baseline in QLF score at month 6 were 11.4% in the nintedanib group (n=42) and 14.6% in the placebo group (n=45) (difference 3.2% [95% CI: −9.2, 15.6]). Adjusted mean absolute changes from baseline in QLF score at month 6 were 0.98% and 1.33% in these groups, respectively (difference 0.35% [95% CI: −1.27, 1.96]). Adjusted mean absolute changes from baseline in FVC at month 6 were −14.2 mL and −83.2 mL in the nintedanib (n=54) and placebo (n=54) groups, respectively (difference 69.0 mL [95% CI: −8.7, 146.8]). Conclusion: Exploratory data suggest that in patients with IPF, 6 months’ treatment with nintedanib was associated with a numerically smaller degree of fibrotic change in the lungs and reduced FVC decline versus placebo. These data support previous findings that nintedanib slows the progression of IPF.

scholarly journals Differential long non-coding RNA expression profile and function analysis in primary Sjogren’s syndrome

2021 ◽  
Author(s):  
Xiaochan Chen ◽  
Qi Cheng ◽  
Yan Du ◽  
Lei Liu ◽  
Huaxiang Wu
Keyword(s):  
B Cells ◽  
Primary Sjögren’S Syndrome ◽  
Differentially Expressed ◽  
Expression Level ◽  
Primary Sjogren’S Syndrome ◽  
Primary Sjögren's Syndrome ◽  
Cerna Network ◽  
Non Coding Rna ◽  
Long Non Coding Rna ◽  
Healthy Persons

Abstract Background: Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disease characterized by abnormal immune cell activation. This study aimed to investigate differentially expressed long non-coding RNA (lncRNA) in peripheral blood mononuclear cells (PBMCs) in patients with pSS to identify lncRNAs that affect pSS pathogenesis. Methods: Total RNA was extrated from PBMCs of 30 patients with pSS and 15 healthy persons. Transcriptome sequencing was used to screen differentially expressed lncRNAs and mRNAs in 8 RNA samples from the discovery cohort. The differentially expressed mRNAs underwent functional enrichment analysis. A protein interaction relationship (PPI) and ceRNA network was constructed. Real-time PCR was used to validate screened lncRNAs in all 45 RNA samples. Results: 1180 lncRNAs and 640 mRNAs were differentially expressed in pSS patients (fold change > 2 in healthy persons). The PPI network was constructed with 640 mRNAs and a ceRNA network with four key lncRNAs (GABPB1-AS1, PSMA3-AS1, LINC00847 and SNHG1). RT-PCR revealed that GABPB1-AS1 and PSMA3-AS1 were significantly upregulated 3.0-and 1.4-fold in the pSS group, respectively. The GABPB1-AS1 expression level was positively correlated with the percentage of B cells and IgG levels. Conclusions: GABPB1-AS1 was significently upregulated in pSS patients, and its expression level is positively correlated with the percentage of B cells and IgG levels. GABPB1-AS1 may be involved in the pathogenesis of pSS.

Sign in / Sign up

Export Citation Format

Share Document