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interstitial pulmonary fibrosis

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https://www.readbyqxmd.com/read/29667101/clinical-features-risk-factors-and-outcomes-of-patients-with-interstitial-pneumonia-with-autoimmune-features-a-population-based-study
#1
Jinghong Dai, Lei Wang, Xin Yan, Hui Li, Kefeng Zhou, Jian He, Fanqing Meng, Siyi Xu, Geyu Liang, Hourong Cai
To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint...
April 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29649237/bronchoalveolar-lavage-bal-cells-in-idiopathic-pulmonary-fibrosis-express-a-complex-pro-inflammatory-pro-repair-angiogenic-activation-pattern-likely-associated-with-macrophage-iron-accumulation
#2
Jungnam Lee, Ivan Arisi, Ermanno Puxeddu, Lazarus K Mramba, Massimo Amicosante, Carmen M Swaisgood, Marco Pallante, Mark L Brantly, C Magnus Sköld, Cesare Saltini
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown cause characterized by alveolar epithelial damage, patchy interstitial fibrosis and diffuse microvascular abnormalities. In IPF, alveolar clustering of iron-laden alveolar macrophages-a common sign of microhemorrhage, has been associated with vascular abnormalities and worsening of pulmonary hypertension. As iron-dependent ROS generation has been shown to induce unrestrained macrophage activation in disease models of vascular damage, we explored alveolar macrophage activation phenotype in IPF patients (n = 16) and healthy controls (CTR, n = 7) by RNA sequencing of bronchoalveolar lavage (BAL) cells...
2018: PloS One
https://www.readbyqxmd.com/read/29642520/melatonin-protects-against-lung-fibrosis-by-regulating-the-hippo-yap-pathway
#3
Xiaoguang Zhao, Jian Sun, Wei Su, Huitong Shan, Bowen Zhang, Yining Wang, Azaliia Shabanova, Hongli Shan, Haihai Liang
Idiopathic pulmonary fibrosis (IPF) is a progressive, fibrotic interstitial pneumonia with high mortality. Melatonin, a hormone predominantly secreted by the pineal gland, has been reported to participate in the process of IPF. However, the mechanisms underlying the effect of melatonin in pulmonary fibrosis have not been elucidated to date. This study was designed to evaluate the anti-fibrotic role of melatonin in pulmonary fibrosis and to elucidate the potential mechanisms. We observed that melatonin markedly attenuated bleomycin (BLM)-induced experimental lung fibrosis in mice and inhibited TGF-β1-induced fibrogenesis in lung fibroblasts...
April 9, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29627072/burden-resilience-and-coping-in-caregivers-of-patients-with-interstitial-lung-disease
#4
R J Shah, H R Collard, J Morisset
RATIONALE: Prior work has described the experience of caregiving in idiopathic pulmonary fibrosis, but the effect on caregivers in interstitial lung disease (ILD) has not been explored. OBJECTIVES: Describe the burden, resilience, and health related quality of life (HRQoL) of caregivers of people with ILD. METHODS: In a mixed methods study, ILD caregivers completed questionnaires and participated in focus groups. A qualitative thematic analysis of the focus group transcripts was conducted...
April 4, 2018: Heart & Lung: the Journal of Critical Care
https://www.readbyqxmd.com/read/29620190/lncrnapcat29-inhibits-pulmonary-fibrosis-via-the-tgf%C3%A2-%C3%AE-1%C3%A2-regulated-rasal1-erk1-2-signal-pathway
#5
Xiaoming Liu, Shanyu Gao, Huile Xu
Pulmonary fibrosis is a severe respiratory disease characterized by the aggregation of extracellular matrix components and inflammation‑associated injury. Studies have suggested that long non‑coding RNAs (lncRNA) may serve a role in the pathophysiological processes of pulmonary fibrosis. However, the potential molecular mechanisms involving the lncRNA, prostate cancer‑associated transcript 29 (lncRNAPCAT29) in the progression of pulmonary fibrosis are yet to be determined. In the present study, the role of lncRNAPCAT29 and the potential signaling mechanism in pulmonary fibrosis progression was investigated...
March 28, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29607151/lung-cancer-in-connective-tissue-disease-associated-interstitial-lung-disease-clinical-features-and-impact-on-outcomes
#6
Satoshi Watanabe, Keigo Saeki, Yuko Waseda, Akari Murata, Hazuki Takato, Yukari Ichikawa, Masahide Yasui, Hideharu Kimura, Yasuhito Hamaguchi, Takashi Matsushita, Kazunori Yamada, Mitsuhiro Kawano, Kengo Furuichi, Takashi Wada, Kazuo Kasahara
Backgrounds: Lung cancer (LC) adversely impacts survival in patients with idiopathic pulmonary fibrosis. However, little is known about LC in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). The aim of this study was to evaluate the prevalence of and risk factors for LC in CTD-ILD, and the clinical characteristics and survival of CTD-ILD patients with LC. Methods: We conducted a single-center, retrospective review of patients with CTD-ILD from 2003 to 2016...
February 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29606857/the-reliability-of-lung-ultrasound-in-assessment-of-idiopathic-pulmonary-fibrosis
#7
REVIEW
Diana Manolescu, Lavinia Davidescu, Daniel Traila, Cristian Oancea, Voicu Tudorache
Idiopathic pulmonary fibrosis (IPF) is the severest form of idiopathic interstitial pneumonia, with a median survival time estimated at 2-5 years from the time of diagnosis. It occurs mainly in elderly adults, suggesting a strong link between the fibrosis process and aging. Although chest high-resolution computed tomography (HRCT) is currently the method of choice in IPF assessment, diagnostic imaging with typical usual interstitial pneumonia (UIP) provides definitive results in only 55%, requiring an invasive surgical procedure such as lung biopsy or cryobiopsy for the final diagnostic analysis...
2018: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/29605213/interstitial-lung-abnormality-is-prevalent-and-associated-with-worse-outcome-in-patients-undergoing-transcatheter-aortic-valve-replacement
#8
Michael Kadoch, Aleksandar Kitich, Shehabaldin Alqalyoobi, Elyse Lafond, Elena Foster, Maya Juarez, Cesar Mendez, Thomas W Smith, Garrett Wong, Walter D Boyd, Jeffrey Southard, Justin M Oldham
BACKGROUND: Interstitial lung abnormality (ILA) is found in 5-10% of the general population and is associated with increased mortality risk. Risk factors for ILA, including advanced age and smoking history also increase the risk for aortic stenosis (AS). Transcatheter aortic valve replacement (TAVR) has become an increasingly utilized intervention for patients with severe AS, and requires a high-resolution computed tomography (HRCT) of the chest to assess aortic valve dimensions. OBJECTIVES: To determine the prevalence and clinical significance of ILA on HRCT performed in patients referred for TAVR...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29605201/distinctive-characteristics-and-prognostic-significance-of-interstitial-pneumonia-with-autoimmune-features-in-patients-with-chronic-fibrosing-interstitial-pneumonia
#9
Katsuhiro Yoshimura, Masato Kono, Yasunori Enomoto, Koji Nishimoto, Yoshiyuki Oyama, Hideki Yasui, Hironao Hozumi, Masato Karayama, Yuzo Suzuki, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Yutaro Nakamura, Naoki Inui, Hiromitsu Sumikawa, Takeshi Johkoh, Thomas V Colby, Haruhiko Sugimura, Takafumi Suda
BACKGROUND: Interstitial lung diseases are heterogeneous, and patients with chronic fibrosing interstitial pneumonia (CFIP) often have clinical, serologic, and morphologic features suggestive but not diagnostic of connective tissue disease. Recently, the concept of interstitial pneumonia with autoimmune features (IPAF) has been proposed as a platform for such patients. However, the prognostic role of IPAF, including the cumulative incidence of acute exacerbations (AEs), is not fully clear...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29604596/submicron-emulsion-of-cinnamaldehyde-ameliorates-bleomycin-induced-idiopathic-pulmonary-fibrosis-via-inhibition-of-inflammation-oxidative-stress-and-epithelial-mesenchymal-transition
#10
Li Yan, Fan Song, Hua Li, Yao Li, Jie Li, Qiao-Yan He, Di Zhang, Fang Wang, Meng Zhang, Hang Zhao, Tian Feng, Ying-Yong Zhao, Si-Wang Wang
AIMS: Idiopathic pulmonary fibrosis (IPF) is the most frequent and severe form of idiopathic interstitial pneumonias. The pathogenesis is associated with inflammation and oxidative stress and epithelial-mesenchymal transition (EMT). Cinnamaldehyde exhibits antiinflammatory and antioxidant properties, but its effect on IPF is unknown. The present study is to investigate the anti-fibrotic effect and action mechanism of cinnamaldehyde on IPF. MATERIALS AND METHODS: IPF was induced by intratracheal bleomycin in mice...
March 28, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29601564/-nintedanib-in-the-treatment-of-fibrosing-interstital-lung-diseases
#11
REVIEW
Katarzyna Lewandowska
Nintedanib is an intracellular tyrosine kinase inhibitor approved in a treatment of idiopathic pulmonary fibrosis. It reduces the annual rate of forced vital capacity decline by approximately 50%, that results in slowing of disease progression. The drug also reduces the incidence of acute exacerbations of idiopathic pulmonary fibrosis. The efficacy of nintedanib was the same in different groups of patients, irrespectively to disease status, age, gender and race. The most frequent side effects were gastrointestinal, i...
March 27, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#12
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29590152/clinical-spectrum-and-prognostic-factors-of-possible-uip-pattern-on-high-resolution-ct-in-patients-who-underwent-surgical-lung-biopsy
#13
Yasuhiro Kondoh, Hiroyuki Taniguchi, Kensuke Kataoka, Taiki Furukawa, Ayumi Shintani, Tomoyuki Fujisawa, Takafumi Suda, Machiko Arita, Tomohisa Baba, Kazuya Ichikado, Yoshikazu Inoue, Kazuma Kishi, Tomoo Kishaba, Osamu Nishiyama, Takashi Ogura, Keisuke Tomii, Sakae Homma
BACKGROUND: Few studies have reported the diagnostic variability in patients with a possible usual interstitial pneumonia (UIP) pattern on high-resolution CT (HRCT) who underwent surgical lung biopsy (SLB), and the prognostic factors for these patients have not been fully evaluated. We retrospectively investigated the frequency of idiopathic pulmonary fibrosis (IPF) and prognostic factors in patients with possible UIP pattern on HRCT. METHODS: Consecutive patients who had a possible UIP pattern on HRCT, underwent SLB, and had a diagnosis of IIPs before SLB were retrospectively recruited from 10 hospitals...
2018: PloS One
https://www.readbyqxmd.com/read/29587761/evaluation-of-oxidative-stress-biomarkers-in-idiopathic-pulmonary-fibrosis-and-therapeutic-applications-a-systematic-review
#14
REVIEW
Alessandro G Fois, Panagiotis Paliogiannis, Salvatore Sotgia, Arduino A Mangoni, Elisabetta Zinellu, Pietro Pirina, Ciriaco Carru, Angelo Zinellu
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF), a fatal lung disease of unknown origin, is characterized by chronic and progressive fibrosing interstitial pneumonia which progressively impairs lung function. Oxidative stress is one of the main pathogenic pathways in IPF. The aim of this systematic review was to describe the type of markers of oxidative stress identified in different biological specimens and the effects of antioxidant therapies in patients with IPF. METHODS: We conducted a systematic search of publications listed in electronic databases (Pubmed, Web of Science, Scopus and Google Scholar) from inception to October 2017...
March 27, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29587263/a-real-life-multicenter-national-study-on-nintedanib-in-severe-idiopathic-pulmonary-fibrosis
#15
Sergio Harari, Antonella Caminati, Venerino Poletti, Marco Confalonieri, Stefano Gasparini, Donato Lacedonia, Fabrizio Luppi, Alberto Pesci, Alfredo Sebastiani, Paolo Spagnolo, Carlo Vancheri, Elisabetta Balestro, Martina Bonifazi, Stefania Cerri, Federica De Giacomi, Rossana Della Porta, Maria Pia Foschino Barbaro, Annalisa Fui, Patrizio Pasquinelli, Roberta Rosso, Sara Tomassetti, Claudia Specchia, Paola Rottoli
BACKGROUND: Two therapeutic options are currently available for patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. To date, there is still insufficient data on the efficacy of these 2 agents in patients with more severe disease. OBJECTIVES: This national, multicenter, retrospective real-life study was intended to determine the impact of nintedanib on the treatment of patients with severe IPF. METHODS: All patients included had severe IPF and had to have at least 6 months of follow-up before and at least 6 months of follow-up after starting nintedanib...
March 27, 2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29563341/active-epithelial-hippo-signaling-in-idiopathic-pulmonary-fibrosis
#16
Jason J Gokey, Anusha Sridharan, Yan Xu, Jenna Green, Gianni Carraro, Barry R Stripp, Anne-Karina T Perl, Jeffrey A Whitsett
Hippo/YAP signaling plays pleiotropic roles in the regulation of cell proliferation and differentiation during organogenesis and tissue repair. Herein we demonstrate increased YAP activity in respiratory epithelial cells in lungs of patients with idiopathic pulmonary fibrosis (IPF), a common, lethal form of interstitial lung disease (ILD). Immunofluorescence staining in IPF epithelial cells demonstrated increased nuclear YAP and loss of MST1/2. Bioinformatic analyses of epithelial cell RNA profiles predicted increased activity of YAP and increased canonical mTOR/PI3K/AKT signaling in IPF...
March 22, 2018: JCI Insight
https://www.readbyqxmd.com/read/29555917/comparison-of-cpi-and-gap-models-in-patients-with-idiopathic-pulmonary-fibrosis-a-nationwide-cohort-study
#17
Sang Hoon Lee, Jong Sun Park, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik Park, Sung Woo Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Moo Suk Park
The clinical course of idiopathic pulmonary fibrosis (IPF) is difficult to predict, partly owing to its heterogeneity. Composite physiologic index (CPI) and gender-age-physiology (GAP) models are easy-to-use predictors of IPF progression. This study aimed to compare the predictive values of these two models. From 2003 to 2007, the Korean Interstitial Lung Disease (ILD) Study Group surveyed ILD patients using the 2002 ATS/ERS criteria. A total of 832 patients with IPF were enrolled in this study. CPI was calculated as follows: 91...
March 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29547052/ct-pathologic-correlation-of-major-types-of-pulmonary-fibrosis-insights-for-revisions-to-current-guidelines
#18
Jonathan H Chung, Justin M Oldham, Steven M Montner, Rekha Vij, Ayodeji Adegunsoye, Aliya N Husain, Imre Noth, David A Lynch, Mary E Strek
OBJECTIVE: The purpose of this study was to assess the diagnostic significance of CT patterns that cannot be classified according to current idiopathic pulmonary fibrosis (IPF) guidelines and of specific findings of the inconsistent with usual interstitial pneumonitis (UIP) pattern. MATERIALS AND METHODS: Subjects with a multidisciplinary diagnosis of interstitial lung disease who had undergone surgical lung biopsy and chest CT within 1 year of each other were included in the study...
March 16, 2018: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/29543042/nintedanib-reduces-pulmonary-fibrosis-in-a-model-of-rheumatoid-arthritis-associated-interstitial-lung-disease
#19
Elizabeth F Redente, Martin A Aguilar, Bart P Black, Benjamin Edelman, Ali Bahadur, Stephen M Humphries, David A Lynch, Lutz Wollin, David W H Riches
Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) develops in approximately 20% of patients with RA. SKG mice, which are genetically prone to developing autoimmune arthritis, develop a pulmonary interstitial pneumonia that resembles human cellular and fibrotic non-specific interstitial pneumonia. Nintedanib, a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis, has been shown to reduce lung function decline. Therefore, we investigated the effect of nintedanib on the development of pulmonary fibrosis and joint disease in female SKG mice with arthritis induced by intraperitoneal injection of zymosan (5 mg)...
March 15, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29541474/early-diagnosis-of-idiopathic-pulmonary-haemosiderosis-increased-haemosiderin-laden-macrophages-in-repeat-bronchoscopy
#20
Yuichi Mukai, Toshihiko Agatsuma, Gen Ideura
Idiopathic pulmonary haemosiderosis (IPH) is a diagnosis of exclusion, which is characterized by persistent or recurrent episodes of alveolar haemorrhage. Early diagnosis of IPH, especially in the case of first-time manifestation, is challenging because previous episodes of alveolar haemorrhage are often difficult to prove. Repeated episodes of alveolar haemorrhage can result in chronic iron-deficient anaemia and irreversible interstitial fibrosis; thus, early recognition and intervention are desirable in terms of clinical outcome...
April 2018: Respirology Case Reports
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