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154 papers 25 to 100 followers
Choua Thao, Amir Lagstein, Tadashi Allen, Huseyin Erhan Dincer, Hyun Joo Kim
Respiratory involvement in Crohn's disease (CD) is a rare manifestation known to involve the large and small airways, lung parenchyma, and pleura. The clinical presentation is nonspecific, and diagnostic tests can mimic other pulmonary diseases, posing a diagnostic challenge and delay in treatment. We report a case of a 60-year-old female with a history of CD and psoriatic arthritis who presented with dyspnea, fever, and cough with abnormal radiological findings. Diagnostic testing revealed an elevated CD4:CD8 ratio in the bronchoalveolar lavage fluid, and cryoprobe lung biopsy results showed non-necrotizing granulomatous inflammation...
October 7, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Maria Paula Henao, Timothy J Craig
Alpha-1-antitrypsin Deficiency (AATD) is an orphan disease that predisposes individuals to COPD and liver disease. The following is a comprehensive review of AATD from epidemiology to treatment for physicians who treat COPD or asthma. Areas Covered: In this comprehensive review of alpha-1-antitrypsin deficiency, we describe the historical perspective, genetics, epidemiology, clinical presentation and symptoms, screening and diagnosis, and treatments of the condition. Expert Commentary: The two most important directions for advancing the understanding of AATD involve improving detection of the condition, especially in asymptomatic patients, and advancing knowledge of treatments directed specifically at AATD-related conditions...
October 24, 2016: Expert Review of Respiratory Medicine
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
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
Jamie S Sheth, John A Belperio, Michael C Fishbein, Ella A Kazerooni, Amir Lagstein, Susan Murray, Jeff L Myers, Richard H Simon, Thomas H Sisson, Baskaran Sundaram, Eric S White, Meng Xia, David Zisman, Kevin R Flaherty
BACKGROUND: Surgical lung biopsy (SLB) is invasive and not possible in all patients with undiagnosed interstitial lung diseases (ILD). We hypothesized that transbronchial biopsy (TBB) findings combined with clinical and high resolution computed tomography (HRCT) data leads to a confident diagnosis congruent to SLB, and therefore avoids the need for SLB in some patients. METHODS: We evaluated 33 patients being evaluated for suspected ILD who underwent HRCT, TBB and SLB...
October 8, 2016: Chest
Andrea Picchianti Diamanti, Milica Markovic, Giuseppe Argento, Simonetta Giovagnoli, Alberto Ricci, Bruno Laganà, Raffaele D'Amelio
Rheumatoid arthritis (RA) is an inflammatory autoimmune disease that can present different extrarticular manifestations involving heart, lungs and kidneys. In recent years there has been a growing awareness of the central role played by the lungs in the onset and progression of RA. In particular interstitial lung disease (ILD) is a common pulmonary manifestation that may be related to the inflammatory process itself, infectious complications and to the treatments used. Management of patients with ILD/RA is still a challenge for clinicians, both synthetic [mainly methotrexate (MTX), leflunomide] and biologic immunosuppressors [mainly anti-tumor necrosis factor (TNF)α] have in fact been related to the onset or worsening of lung diseases with conflicting data...
October 12, 2016: Therapeutic Advances in Respiratory Disease
Antonella Arcadu, Teng Moua
BACKGROUND AND OBJECTIVE: Acute respiratory failure (ARF) in patients with interstitial lung disease (ILD) is associated with significant morbidity and mortality. Recommended assessment for acute exacerbation (AE) of ILD includes exclusion of secondary causes via fibreoptic bronchoscopy. Our aim is to assess the role of bronchoscopy during ARF-ILD. METHODS: Consecutive ILD patients (2002-2014) hospitalized with ARF who underwent bronchoscopy were included. Baseline demographics, underlying ILD diagnoses and presenting clinical features were reviewed...
October 6, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
Giorgia A Osman, Alberto Ricci, Fabrizio Terzo, Carlo Falasca, Maria R Giovagnoli, Pierdonato Bruno, Andrea Vecchione, Salvatore Raffa, Sabatino Valente, Maria R Torrisi, Chiara De Dominicis, Simonetta Giovagnoli, Salvatore Mariotta
INTRODUCTION: Lipoid pneumonia is a clinical condition that may be initially asymptomatic or confused with an infectious or malignant lung disease. OBJECTIVES: We report four cases of this pathological condition. METHODS: The first case concerned an 85-year old woman with bilateral confluent pulmonary opacities, ground-glass type. Diagnosis was based on the cytology of the bronchoalveolar lavage (BAL) fluid followed by its ultrastructural examination...
September 22, 2016: Clinical Respiratory Journal
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
Christina Paul, Ammy Lin-Shaw, Mariamma Joseph, Keith Kwan, Gianluigi Sergiacomi, Marco Mura
Organising pneumonia (OP) is usually promptly responsive to corticosteroid treatment. We describe a series of 3 cases of severe, progressive, biopsy-proven fibrosing OP causing respiratory failure. All cases presented with peribronchial and subpleural consolidations, had a fibro-inflammatory infiltrative component in the alveolar septa, and only had a partial and unsatisfactory response to corticosteroids. However, they responded to mycophenolic acid (MPA) treatment with resolution of respiratory failure as well as clinical and functional improvement...
2016: Respiration; International Review of Thoracic Diseases
Masashi Bando
Pirfenidone is an oral drug that exerts not only anti-fibrotic activity but also pleiotropic effects, such as anti-inflammatory and anti-oxidative effects. Because it suppresses reduction in vital capacity and improves progression-free survival, it was approved in October 2008 in Japan for the first time in the world as an anti-fibrotic agent for treatment of idiopathic pulmonary fibrosis (IPF). In October 2014, the agent was approved in the U.S., based on the results of the ASCEND study. Today, it is commercially available in 38 countries worldwide...
September 2016: Respiratory Investigation
Martin Kolb, Luca Richeldi, Jürgen Behr, Toby M Maher, Wenbo Tang, Susanne Stowasser, Christoph Hallmann, Roland M du Bois
RATIONALE: There is no consensus as to when treatment for idiopathic pulmonary fibrosis (IPF) should be initiated. Some physicians prefer not to treat patients with preserved lung volume. OBJECTIVE: To investigate whether patients with IPF and preserved lung volume receive the same benefit from nintedanib as patients with more impaired lung volume. METHODS: Post hoc subgroup analyses of pooled data from the two replicate phase III INPULSIS trials by baseline FVC % predicted (≤90%, >90%)...
September 26, 2016: Thorax
John P Hutchinson, Tricia M McKeever, Andrew W Fogarty, Vidya Navaratnam, Richard B Hubbard
International guidelines and new targeted therapies for idiopathic pulmonary fibrosis have increased the need for accurate diagnosis of interstitial lung disease (ILD), which may lead to more surgical lung biopsies. This study aimed to assess the risk of this procedure in patients from the UK.We used Hospital Episodes Statistics data from 1997 to 2008 to assess the frequency of surgical lung biopsy for ILD in England, UK. We identified cardiothoracic surgical patients using International Classification of Diseases revision 10 codes for ILD and Office of Population Censuses and Surveys Classification of Interventions and Procedures version 4 codes for surgical lung biopsy...
September 22, 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Kamonpun Ussavarungsi, Ryan M Kern, Anja C Roden, Jay H Ryu, Eric S Edell
BACKGROUND: Diagnostic evaluation of patients with diffuse parenchymal lung disease (DPLD) is best achieved by a multidisciplinary team correlating clinical, radiologic, and pathologic features. Surgical lung biopsies remain the gold standard for histopathologic diagnosis of idiopathic interstitial pneumonias. Emerging data suggest an increasing role for transbronchial cryobiopsy (TBC) in DPLD evaluation. We describe our experience with TBC in patients with DPLD. METHODS: We retrospectively reviewed medical records of patients with radiographic features of DPLD who underwent TBC at Mayo Clinic, Rochester, Minnesota in June 2013-September 2015...
September 19, 2016: Chest
Yutaro Nakamura, Takafumi Suda
Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF...
2015: Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine
Christopher S King, Steven D Nathan
Despite the development of pharmacological therapies that are effective in slowing the progression of idiopathic pulmonary fibrosis (IPF), it remains a debilitating and lethal condition. In addition to the adverse effects caused by pulmonary fibrosis, most patients with IPF have associated comorbid conditions, which might negatively affect functional status, quality of life, and survival. Comorbid conditions can be pulmonary or extrapulmonary. Pulmonary comorbidities include pulmonary hypertension, emphysema, and lung cancer, while non-pulmonary conditions include venous thromboembolism, coronary artery disease, congestive heart failure, sleep-disordered breathing, gastro-oesophageal reflux disease, and anxiety or depression...
September 2, 2016: Lancet Respiratory Medicine
Kam Yu Chiu, Jian Guo Li, Ying Ying Gu
Acute fibrinous and organizing pneumonia (AFOP) is an uncommon variant of acute lung injury. Here, we report a patient with AFOP and a previously unreported condition, pneumothorax. After our experience with this case, we suggest that exposure to birds may be associated with AFOP; pneumothorax can develop in patients with AFOP; and glucocorticoids are very effective for treating AFOP.
September 10, 2016: Clinical Respiratory Journal
Mirjam J G van Manen, Surinder S Birring, Carlo Vancheri, Vincent Cottin, Elisabetta A Renzoni, Anne-Marie Russell, Marlies S Wijsenbeek
Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably "multifactorial" and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF...
September 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
Elizabeth R Volkmann, Donald P Tashkin
Although interstitial lung disease accounts for the majority of deaths in patients with systemic sclerosis, treatment options for this manifestation of systemic sclerosis are limited. Few high quality, randomized controlled trials exist for systemic sclerosis-related interstitial lung disease, and historically, studies have favored the use of cyclophosphamide. However, the benefit of cyclophosphamide for systemic sclerosis-related interstitial lung disease is tempered by its complex adverse event profile. More recent studies have demonstrated the effectiveness of mycophenolate for systemic sclerosis-related interstitial lung disease, including the recently published Scleroderma Lung Study II...
August 25, 2016: Annals of the American Thoracic Society
Oksana A Shlobin, A Whitney Brown, Steven D Nathan
Pulmonary hypertension (PH) can be triggered by any number of disease processes that result in increased pulmonary vascular resistance. Although historically associated with idiopathic pulmonary arterial hypertension (iPAH), the majority of patients with PH do not have the idiopathic subtype, but rather PH associated with another underlying diagnosis, such as left heart or lung disease. The World Health Organization (WHO) classification of PH helps conceptualize the different categories based on presumed etiology...
August 20, 2016: Chest
2016-08-26 19:59:19
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