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interstitial pulmonary fibrosis

Li Gao, Sheng Xie, Hui Liu, Pingping Liu, Yan Xiong, Jiping Da, Chengli Que, Huaping Dai, Chen Wang
OBJECTIVE: Combined pulmonary fibrosis and emphysema (CPFE) is a newly defined entity that comprises upper lobe emphysema and lower lobe fibrosis. Patients with CPFE are at high risk for lung cancer and have poor prognoses. To investigate the clinical and pathological characteristics of lung cancer with CPFE, lung cancers with CPFE and Non-CPFE interstitial lung disease (ILD) were reevaluated by 2015 WHO classification and compared. METHODS: A total of 60 patients with histologically proven lung cancer were selected from the database of two institutional medical centers...
October 21, 2016: Clinical Respiratory Journal
Denis E O'Donnell, J Alberto Neder, Ingrid Harle, Onofre Moran-Mendoza
Idiopathic pulmonary fibrosis (IPF) is one of the most common forms of interstitial lung disease, with a median survival time of two to five years. Most patients with IPF experience chronic breathlessness, which is closely linked to poor perceived quality of life and significant restriction of daily activities; therefore, effective management of this distressing symptom is a major goal of patient care. Areas covered: This report summarizes the physiology of IPF during rest and exercise, outlines current concepts of the mechanisms of breathlessness, and provides a physiological rationale for optimal management of individual patient...
October 21, 2016: Expert Review of Respiratory Medicine
Martina Doubková, Michal Karpíšek, Jiri Mazoch, Jana Skřičková, Michael Doubek
BACKGROUND: Identification of serum and bronchoalveolar lavage fluid (BALF) biomarkers may facilitate diagnosis and prognostication in various lung disorders. OBJECTIVE: Serum and BALF levels of surfactant protein A (SP-A), surfactant protein D (SP-D), Clara cell protein 16 (CC16), S100 protein, trefoil factor 3 (TFF3), and prostatic secretory protein 94 (PSP94) were evaluated in 94 consecutive patients (idiopathic pulmonary fibrosis (IPF; n=18), sarcoidosis (n=25), chronic obstructive pulmonary disease (COPD; n=51)), and in 155 healthy controls...
October 7, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Sang Hoon Lee, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik 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, Sung Woo Park, Moo Suk Park
BACKGROUND: The clinical course of idiopathic pulmonary fibrosis (IPF) varies widely. Although the GAP model is useful for predicting mortality, survivals have not yet been validated for each GAP score. We aimed to elucidate how prognosis is related to GAP score and GAP stage in IPF patients. METHODS: The Korean Interstitial Lung Disease Study Group conducted a national survey to evaluate various characteristics in IPF patients from 2003 to 2007. Patients were diagnosed according to the 2002 criteria of the ATS/ERS...
October 18, 2016: Respiratory Research
Ankit Mangla, Nikki Agarwal, Chou Carmel, Thomas Lad
Erlotinib is one of the most widely used tyrosine kinase inhibitor targeting human epidermal growth factor receptor. Since its introduction, it has revolutionized the treatment of advanced non-small cell lung cancer. Skin rashes and diarrhea are the most often reported side effects of erlotinib however it is also associated with interstitial pneumonitis or interstitial lung disease, which often turns out to be fatal complication of using this medicine. Though reported scarcely in the western world, the association of interstitial lung disease with epidermal growth factor receptor has attracted a lot of attention in the recent times...
September 5, 2016: Rare Tumors
Jun Fukihara, Yasuhiro Kondoh
Nintedanib is a new anti-fibrosis agent that is an intracellular tyrosine kinase inhibitor targeting platelet derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor. Although nintedanib is attracting much attention as a new treatment option for patients with idiopathic pulmonary fibrosis (IPF), the clinical evidence is limited mainly to the results from the dose-deciding phase II TOMORROW trial and phase III INPULSIS trials, which evaluated efficacy and safety of nintedanib for patients with IPF, prespecified subgroup analyses, pooled analyses and meta-analyses derived from those trials...
October 17, 2016: Expert Review of Respiratory Medicine
K P Suraj, Neethu K Kumar, E Jyothi, Kiran Vishnu Narayan, G Biju
BACKGROUND: But so far there is no proven pharmacological treatment for Idiopathic pulmonary fibrosis (IPF). As trials investigating different agents with different mechanisms of actions are going on, encouraging results have led to the licensing of the first IPF-specific drug, Pirfenidone. OBJECTIVE: To assess the proportion of IPF among interstitial lung disease patients and to assess their treatment response to Pirfenidone. MATERIAL AND METHODS: All consecutive patients attending the outpatient department from 1st January 2012 to 30th June 2012 with a proven diagnosis of Interstitial lung Disease (ILD) were included in this longitudinal cohort study...
May 2016: Journal of the Association of Physicians of India
Elisabetta Fenocchio, Ilaria Depetris, Delia Campanella, Lucia Garetto, Fabrizio Carnevale Schianca, Danilo Galizia, Giovanni Grignani, Massimo Aglietta, Francesco Leone
BACKGROUND: Gemcitabine is currently the standard chemotherapy for the adjuvant treatment of pancreatic cancer. This chemotherapeutic agent is generally well-tolerated, myelosuppression and gastrointestinal toxicity being common side effects. Nevertheless, gemcitabine-induced pulmonary toxicity has been rarely reported. Despite its low incidence, the spectrum of pulmonary injury is wide, including potentially fatal conditions. We report a case of acute interstitial pneumonia related to gemcitabine, completely solved with Imatinib Mesylate (IM)...
October 12, 2016: BMC Cancer
Chongxiang Xiong, Monica V Masucci, Xiaoxu Zhou, Na Liu, Xiujuan Zang, Evelyn Tolbert, Ting C Zhao, Shougang Zhuang
Bromodomain and extra-terminal (BET) protein inhibitors have been shown to effectively inhibit tumorgenesis and ameliorate pulmonary fibrosis by targeting bromodomain proteins that bind acetylated chromatin markers. However, their pharmacological effects in renal fibrosis remain unclear. In this study, we examined the effect of I-BET151, a selective and potent BET inhibitor, on renal fibroblast activation and renal fibrosis. In cultured renal interstitial fibroblasts, exposure of cells to I-BET151, or silencing of bromodoma in-containing protein 4 (Brd4), a key BET protein isoform, significantly reduced their activation as indicated by decreased expression of α-smooth muscle actin, collagen 1 and fibronectin...
October 6, 2016: Oncotarget
Margaret L Wilsher, Lisa M Young, Raewyn Hopkins, Megan Cornere
BACKGROUND AND OBJECTIVE: Ethnicity is strongly associated with variable clinical presentation in sarcoidosis but the association between ethnicity and clinical characteristics has not previously been described in patients of Polynesian ancestry, Maori and Pacific Islander (PI). The objective of this study was to describe the clinical characteristics of sarcoidosis in Maori and PI patients and determine if those were different to European patients. METHODS: A retrospective review of the medical records of 406 patients (69 Maori/PI) attending a specialist interstitial lung disease (ILD) clinic...
October 12, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
Joanna E Kusmirek, Maria Daniela Martin, Jeffrey P Kanne
Idiopathic interstitial pneumonias are a heterogeneous group of diffuse lung diseases characterized by distinct clinicopathologic entities with the usual interstitial pneumonia (UIP) being the most common. The pattern of UIP can be seen in idiopathic pulmonary fibrosis (IPF) as well as in secondary causes, most commonly in connective tissue diseases. IPF is usually progressive and associated with a very poor prognosis, and newer therapies pose a risk of serious complications; therefore, diagnostic certainty is crucial...
November 2016: Radiologic Clinics of North America
Seth Kligerman, Teri J Franks, Jeffrey R Galvin
The direct toxicity of cigarette smoke and the body's subsequent response to this lung injury leads to a wide array of pathologic manifestations and disease states that lead to both reversible and irreversible injury to the large airways, small airways, alveolar walls, and alveolar spaces. These include emphysema, bronchitis, bronchiolitis, acute eosinophilic pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis, desquamative interstitial pneumonia, and pulmonary fibrosis. Although these various forms of injury have different pathologic and imaging manifestations, they are all part of the spectrum of smoking-related diffuse parenchymal lung disease...
November 2016: Radiologic Clinics of North America
Elisa Adele Colombo, Luigina Spaccini, Ludovica Volpi, Gloria Negri, Davide Cittaro, Dejan Lazarevic, Salvatore Zirpoli, Andrea Farolfi, Cristina Gervasini, Maria Vittoria Cubellis, Lidia Larizza
BACKGROUND: Integrin α3 (ITGA3) gene mutations are associated with Interstitial Lung disease, Nephrotic syndrome and Epidermolysis bullosa (ILNEB syndrome). To date only six patients are reported: all carried homozygous ITGA3 mutations and presented a dramatically severe phenotype leading to death before age 2 years, from multi-organ failure due to interstitial lung disease and congenital nephrotic syndrome. The involvement of skin and cutaneous adnexa was variable with sparse hair and nail dysplasia combined or not to skin lesions ranging from skin fragility to epidermolysis bullosa-like blistering...
October 7, 2016: Orphanet Journal of Rare Diseases
Vasilios Tzilas, Demosthenes Bouros
No abstract text is available yet for this article.
October 2016: Lancet Respiratory Medicine
Carlos Ac Pereira, Andréa Gimenez, Lilian Kuranishi, Karin Storrer
Hypersensitivity pneumonitis (HSP) is a common interstitial lung disease resulting from inhalation of a large variety of antigens by susceptible individuals. The disease is best classified as acute and chronic. Chronic HSP can be fibrosing or not. Fibrotic HSP has a large differential diagnosis and has a worse prognosis. The most common etiologies for HSP are reviewed. Diagnostic criteria are proposed for both chronic forms based on exposure, lung auscultation, lung function tests, HRCT findings, bronchoalveolar lavage, and biopsies...
2016: Journal of Asthma and Allergy
Chao Li, Sitong Du, Yiping Lu, Xiaowei Lu, Fangwei Liu, Ying Chen, Dong Weng, Jie Chen
Long term pulmonary exposure to crystalline silica leads to silicosis that manifests progressive interstitial fibrosis, eventually leading to respiratory failure and death. Despite efforts to eliminate silicosis, clinical cases continue to occur in both developing and developed countries. The exact mechanisms of crystalline silica-induced pulmonary fibrosis remain elusive. Herein, we find that 4-1BB is induced in response to crystalline silica injury in lungs and that it is highly expressed during development of experimental silicosis...
2016: Theranostics
Sean J Callahan, Meng Xia, Susan Murray, Kevin R Flaherty
BACKGROUND: A group of patients with idiopathic pulmonary fibrosis (IPF) presents with disease affecting one lung markedly more than the other. At this time, it is unclear how this population differs from those who present with more symmetric disease. We sought to explain the characteristics of the asymmetric group and how their disease progresses. METHODS: In this retrospective case-control study we accessed an interstitial lung disease (ILD) database and identified 14 asymmetric IPF cases via high-resolution computed tomography (HRCT) scoring of each lung lobe's disease severity...
October 2016: Respiratory Medicine
George A Margaritopoulos, Ismini Lasithiotaki, Katerina M Antoniou
Interstitial lung diseases (ILDs) include a number of diseases whose pathogenesis still is not fully understood. Idiopathic pulmonary fibrosis (IPF), the most frequent and severe form of ILDs is an epithelial-driven disease and the treatment consists of the use of antifibrotic agents. In the rest of ILDs an inflammation-driven pathway is believed to be the main pathogenetic mechanism and treatment consists of the use of immunomodulatory agents. In both groups it is believed that infection can play an important role in the development and progression of the diseases...
September 27, 2016: European Journal of Pharmacology
Enas A Hamed, Mostafa M El-Saied, Khaled Saad, Hazem Abu-Zeid Yousef, Amany O Mohamed, Dina Sabry
OBJECTIVE: This study aimed to evaluate fibrosis and elastin destruction in childhood interstitial lung disease (chILD) patients. METHODS: Sixty patients and twenty healthy children were recruited. On admission, evaluation of chILD severity was made using Fan chILD score. Participants provided urine and blood samples. Plasma levels of transforming growth factor (TGF)-β1, connective tissue growth factor (CCN2), soluble factor related apoptosis (sFas) and long non-coding RNAs and urinary levels of desmosine/urinary creatinine (UDes/UCr) were measured...
September 21, 2016: Pathophysiology: the Official Journal of the International Society for Pathophysiology
Sheetu Singh, Bridget F Collins, Bharat B Sharma, Jyotsna M Joshi, Deepak Talwar, Sandeep Katiyar, Nishtha Singh, Lawrence Ho, Jai Kumar Samaria, Parthasarathi Bhattacharya, Rakesh Gupta, Sudhir Chaudhari, Tejraj Singh, Vijay Moond, Sudhakar Pipavath, Jitesh Ahuja, Ravindran Chetambath, Aloke G Ghoshal, Nirmal K Jain, Hj Gayathri Devi, Surya Kant, Parvaiz Koul, Raja Dhar, Rajesh Swarnakar, Suresh Kumar Sharma, Dhrubajyoti J Roy, Kripesh R Sarmah, Bhavin Jankharia, Rodney Schmidt, Santosh K Katiyar, Arpita Jindal, Daya K Mangal, Virendra Singh, Ganesh Raghu
RATIONALE: Interstitial lung disease (ILD) is a heterogeneous group of acute and chronic inflammatory and fibrotic lung diseases. Existing ILD registries have had variable findings. Little is known about the profile of ILDs in India. OBJECTIVES: Create a prospective Registry with validation of diagnoses by multidisciplinary discussion (MDD) to characterize the clinical profile of new onset-ILD in India. METHODS: Prospective recruitment of adult patients with new onset ILD (27 centers in 19 Indian cities, 3/2012-6/2015) with connective tissue disease (CTD) serologies and high-resolution computed tomography (HRCT) chest...
September 29, 2016: American Journal of Respiratory and Critical Care Medicine
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