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https://www.readbyqxmd.com/read/28552544/pulmonary-manifestations-in-the-rheumatic-diseases
#1
REVIEW
Tracy J Doyle, Paul F Dellaripa
Lung disease in the rheumatic diseases presents unique challenges for diagnosis and management and is a source of significant morbidity and mortality for patients. Unlike the idiopathic interstitial pneumonias, patients with rheumatic diseases experience lung disease in the context of a systemic disease that may make it more difficult to recognize and that may present greater risks with treatment. Despite recent advances in our awareness of these diseases, there is still a significant lack of understanding of natural history to elucidate which patients will develop disease that is progressive and thus warrants treatment...
May 25, 2017: Chest
https://www.readbyqxmd.com/read/28472939/chronic-hypersensitivity-pneumonitis-identification-of-key-prognostic-determinants-using-automated-ct-analysis
#2
Joseph Jacob, Brian J Bartholmai, Ryoko Egashira, Anne Laure Brun, Srinivasan Rajagopalan, Ronald Karwoski, Maria Kokosi, David M Hansell, Athol U Wells
BACKGROUND: Chronic hypersensitivity pneumonitis (CHP) has a variable disease course. Computer analysis of CT features was used to identify a subset of CHP patients with an outcome similar to patients with idiopathic pulmonary fibrosis (IPF). METHODS: Consecutive patients with a multi-disciplinary team diagnosis of CHP (n = 116) had pulmonary function tests (FEV1, FVC, DLco, Kco, and a composite physiologic index [CPI]) and CT variables predictive of mortality evaluated by analysing visual and computer-based (CALIPER) parenchymal features: total interstitial lung disease (ILD) extent, honeycombing, reticular pattern, ground glass opacities, pulmonary vessel volume (PVV), emphysema, and traction bronchiectasis...
May 4, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28345369/pulmonary-fibrosis-part-ii-state-of-the-art-patient-management
#3
Keith C Meyer
While many pharmacologic therapies for the treatment of idiopathic pulmonary fibrosis (IPF) have been evaluated via randomized, placebo-controlled clinical trials (RCTs) conducted over the past two decades, most therapies have been shown to be ineffective or even potentially harmful. However, a number of recently completed RCTs have shown significant efficacy for pirfenidone and nintedanib for the treatment of IPF. Areas covered: This manuscript reviews recent advances in the management of IPF and other forms of fibrosing interstitial lung disease (ILD) with an emphasis on IPF...
May 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28345383/pulmonary-fibrosis-part-i-epidemiology-pathogenesis-and-diagnosis
#4
Keith C Meyer
Many forms of interstitial lung disease (ILD) can progress to extensive fibrosis and respiratory failure. Idiopathic pulmonary fibrosis (IPF), which generally has a poor prognosis, has been thoroughly studied over the past two decades, and many important discoveries have been made that pertain to genetic predisposition, epidemiology, disease pathogenesis, diagnosis, and management. Additionally, non-IPF forms of ILD can have radiologic and histopathologic manifestations that mimic IPF, and making an accurate diagnosis is key to providing personalized medicine to patients with pulmonary fibrosis...
May 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28446601/diffuse-parenchymal-lung-disease
#5
REVIEW
Sara Tomassetti, Claudia Ravaglia, Venerino Poletti
Between September 2015 and August 2016 there were >1500 publications in the field of diffuse parenchymal lung diseases (DPLDs). For the Clinical Year in Review session at the European Respiratory Society Congress that was held in London, UK, in September 2016, we selected only five articles. This selection, made from the enormous number of published papers, does not include all the relevant studies that will significantly impact our knowledge in the field of DPLDs in the near future. This review article provides our personal view on the following topics: early diagnosis of idiopathic pulmonary fibrosis, current knowledge on the multidisciplinary team diagnosis of DPLDs and the diagnostic role of transbronchial cryobiopsy in this diagnostic setting, insights on the new entity of interstitial pneumonia with autoimmune features, and new therapeutic approaches for scleroderma-related interstitial lung disease...
June 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28332341/risk%C3%A2-factors-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#6
Meihua Qiu, Yuqing Chen, Qiao Ye
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic disease limited to the lungs. The course of disease varies widely, with some patients experiencing acute respiratory deterioration, a condition called acute exacerbations of IPF (AE-IPF). The risk factors contributing to AE-IPF are unclear. This systematic review and meta-analysis investigated the risk factors for AE-IPF. METHODS: Studies of risk factors for AE-IPF were identified in Medline, EMBASE and Cochrane databases...
March 23, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28286407/pulmonary-hypertension-associated-with-idiopathic-pulmonary-fibrosis-current-and-future-perspectives
#7
REVIEW
Scott D Collum, Javier Amione-Guerra, Ana S Cruz-Solbes, Amara DiFrancesco, Adriana M Hernandez, Ankit Hanmandlu, Keith Youker, Ashrith Guha, Harry Karmouty-Quintana
Pulmonary hypertension (PH) is commonly present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO). PH has been identified to be present in as much as 40% of patients with COPD or IPF and it is considered as one of the principal predictors of mortality in patients with COPD or IPF. However, despite the prevalence and fatal consequences of PH in the setting of chronic lung diseases, there are limited therapies available for patients with Group III PH, with lung transplantation remaining as the most viable option...
2017: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/28225641/a-review-of-clinical-and-imaging-findings-in-eosinophilic-lung-diseases
#8
Adam Bernheim, Theresa McLoud
OBJECTIVE: The purpose of this article is to review the clinical and imaging findings associated with eosinophilic lung diseases. CONCLUSION: The spectrum of eosinophilic lung diseases comprises a diverse group of pulmonary disorders that have an association with tissue or peripheral eosinophilia. These diseases have varied clinical presentations and may be associated with several other abnormalities. Characteristic imaging findings are often detected with chest radiography, and CT best shows parenchymal abnormalities...
May 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28350485/ct-findings-radiologic-pathologic-correlation-and-imaging-predictors-of-survival-for-patients-with-interstitial-pneumonia-with-autoimmune-features
#9
Jonathan H Chung, Steven M Montner, Ayodeji Adegunsoye, Cathryn Lee, Justin M Oldham, Aliya N Husain, Heber MacMahon, Imre Noth, Rekha Vij, Mary E Strek
OBJECTIVE: The objective of this study is to determine the CT findings and patterns of interstitial pneumonia with autoimmune features (IPAF) and to assess whether imaging can predict survival for patients with IPAF. MATERIALS AND METHODS: The study included 136 subjects who met the criteria for IPAF and had diagnostic-quality chest CT scans obtained from 2006 to 2015; a total of 74 of these subjects had pathologic samples available for review within 1 year of chest CT examination...
March 28, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28049126/comorbidities-in-interstitial-lung-diseases
#10
REVIEW
George A Margaritopoulos, Katerina M Antoniou, Athol U Wells
Fibrosing lung disorders include a large number of diseases with diverse behaviour. Patients can die because of the progression of their illness, remain stable or even improve after appropriate treatment has been instituted. Comorbidities, such as acute and chronic infection, gastro-oesophageal reflux, pulmonary hypertension, lung cancer, cardiovascular diseases, and obstructive sleep apnoea, can pre-exist or develop at any time during the course of the disease and, if unidentified and untreated, may impair quality of life, impact upon the respiratory status of the patients, and ultimately lead to disease progression and death...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28096282/the-effect-of-mtor-inhibitors-on-respiratory-infections-in-lymphangioleiomyomatosis
#11
REVIEW
Andrew M Courtwright, Hilary J Goldberg, Elizabeth Petri Henske, Souheil El-Chemaly
Lymphangioleiomyomatosis (LAM) is a destructive cystic lung disease. Mammalian target of rapamycin (mTOR) inhibitors are the primary treatment for LAM but it is unknown whether these immunosuppressing medications increase the risk for or the severity of respiratory infections in LAM patients.We searched multiple databases for original articles that reported the rate of respiratory infections in LAM patients treated with mTOR inhibitors or placebo. We calculated incidence rates for respiratory infections in these groups and incidence rate ratios for respiratory infections and severe respiratory infections in mTOR inhibitors treated versus placebo treated patients...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28096284/pulmonary-vascular-and-cardiac-impairment-in-interstitial-lung-disease
#12
REVIEW
Marios Panagiotou, Alistair C Church, Martin K Johnson, Andrew J Peacock
Pulmonary vascular and cardiac impairment is increasingly appreciated as a major adverse factor in the natural history of interstitial lung disease. This clinically orientated review focuses on the current concepts in the pathogenesis, pathophysiology and implications of the detrimental sequence of increased pulmonary vascular resistance, pre-capillary pulmonary hypertension and right heart failure in interstitial lung disease, and provides guidance on its management.
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28223395/oxygen-therapy-for-interstitial-lung-disease-a-systematic-review
#13
REVIEW
Emily C Bell, Narelle S Cox, Nicole Goh, Ian Glaspole, Glen P Westall, Alice Watson, Anne E Holland
This review aims to establish the impact of oxygen therapy on dyspnoea, health-related quality of life (HRQoL), exercise capacity and mortality in interstitial lung disease (ILD).We included studies that compared oxygen therapy to no oxygen therapy in adults with ILD. No limitations were placed on study design or intervention type. Two reviewers independently evaluated studies for inclusion, assessed risk of bias and extracted data. The primary outcome was dyspnoea.Eight studies evaluated the acute effects of oxygen (n=1509)...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28223398/exertional-dyspnoea-in-interstitial-lung-diseases-the-clinical-utility-of-cardiopulmonary-exercise-testing
#14
REVIEW
Matteo Bonini, Giuseppe Fiorenzano
Interstitial lung diseases (ILDs) represent a heterogeneous group of pathologies characterised by alveolar and interstitial damage, pulmonary inflammation (usually associated with fibrosis), decreased lung function and impaired gas exchange, which can be attributed to either a known or an unknown aetiology. Dyspnoea is one of the most common and disabling symptoms in patients with ILD, significantly impacting quality of life. The mechanisms causing dyspnoea are complex and not yet fully understood. However, it is recognised that dyspnoea occurs when there is an imbalance between the central respiratory efferent drive and the response of the respiratory musculature...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28223152/pathological-findings-and-prognosis-in-a-large-prospective-cohort-of-chronic-hypersensitivity-pneumonitis
#15
Ping Wang, Kirk D Jones, Anatoly Urisman, Brett M Elicker, Thomas Urbania, Kerri A Johannson, Deborah Assayag, Joyce Lee, Paul J Wolters, Harold R Collard, Laura L Koth
BACKGROUND: The ability of specific histopathological features to predict mortality or lung transplant in chronic hypersensitivity pneumonitis patients is unknown. METHODS: Patients with chronic hypersensitivity pneumonitis diagnosed by surgical lung biopsy were identified from an ongoing longitudinal cohort. The surgical lung biopsy slides were evaluated prospectively by an experienced thoracic pathologist using a standardized checklist to differentiate the major pathologic patterns and score the presence of specific histopathological features...
February 18, 2017: Chest
https://www.readbyqxmd.com/read/28222720/birt-hogg-dub%C3%A3-syndrome-a-literature-review-and-case-study-of-a-chinese-woman-presenting-a-novel-flcn-mutation
#16
Shengyu Hao, Fei Long, Fenglan Sun, Teng Liu, Daowei Li, Shujuan Jiang
BACKGROUND: The Birt-Hogg-Dubé (BHD) syndrome is a very rare autosomal dominant form of genodermatosis caused by germline mutations in the folliculin (FLCN) gene, which is mapped to the p11.2 region in chromosome 17. BHD commonly presents cutaneous fibrofolliculomas, pulmonary cysts, renal cell carcinoma, and recurrent pneumothoraxes. The disease is easily ignored or misdiagnosed as pneumothorax, pulmonary lymphangiomyomatosis (LAM), or emphysema. Follow-up and guidelines for managing recurrent pneumothoraxes in these patients are lacking...
February 21, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28099079/lymphangioleiomyomatosis-a-monogenic-model-of-malignancy
#17
Vera P Krymskaya, Francis X McCormack
Lymphangioleiomyomatosis (LAM) is a rare, low-grade, metastasizing neoplasm that arises from an unknown source, spreads via the lymphatics, and targets the lungs. All pulmonary structures become infiltrated with benign-appearing spindle and epithelioid cells (LAM cells) that express smooth-muscle and melanocyte-lineage markers, harbor mTOR-activating mutations in tuberous sclerosis complex (TSC) genes, and recruit abundant stromal cells. Elaboration of lymphangiogenic growth factors and matrix remodeling enzymes by LAM cells enables their access to lymphatic channels and likely drives the cystic lung remodeling that often culminates in respiratory failure...
January 14, 2017: Annual Review of Medicine
https://www.readbyqxmd.com/read/28198728/personalized-medicine-in-interstitial-lung-diseases
#18
Paolo Spagnolo, Justin M Oldham, Mark G Jones, Joyce S Lee
PURPOSE OF REVIEW: A number of recent studies have explored the possibility to apply personalized medicine to interstitial lung diseases (ILDs), particularly idiopathic pulmonary fibrosis (IPF), the most common and deadly of the idiopathic interstitial pneumonias. In our review, we summarize and discuss the most recent literature on personalized medicine in IPF as well as hypersensitivity pneumonitis and sarcoidosis, with emphasis on patient subgroups for which a personalized approach to disease prognostication and management may become a reality in the near future...
May 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28026153/clinical-and-hemodynamic-characteristics-of-pulmonary-hypertension-associated-with-interstitial-lung-disease-in-china
#19
Lan Wang, Qin-Hua Zhao, Bigyan Pudasaini, Rong Jiang, Su-Gang Gong, Jing He, Ping Yuan, Jin-Ming Liu
BACKGROUND: Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD). The aim of the present study was to investigate characteristics and prognosis of ILD associated PH in a predominantly Chinese patient cohort. METHODS: Consecutive patients with ILD associated PH, confirmed by right heart catheterization at shanghai pulmonary hospital were included in the study. Characteristics at baseline and during follow-up, as well as survival, were analyzed...
December 27, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27980445/idiopathic-pulmonary-fibrosis-treatment-and-prognosis
#20
REVIEW
Hajime Fujimoto, Tetsu Kobayashi, Arata Azuma
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a prognosis that can be worse than for many cancers. The initial stages of the condition were thought to mainly involve chronic inflammation; therefore, corticosteroids and other drugs that have anti-inflammatory and immunosuppressive actions were used. However, recently, agents targeting persistent fibrosis resulting from aberrant repair of alveolar epithelial injury have been in the spotlight. There has also been an increase in the number of available antifibrotic treatment options, starting with pirfenidone and nintedanib...
2015: Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine
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