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Idiopathic interstitial pneumonias

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https://www.readbyqxmd.com/read/28817393/increased-expiratory-computed-tomography-density-reveals-possible-abnormalities-in-radiologically-preserved-lung-parenchyma-in-idiopathic-pulmonary-fibrosis
#1
Valentina Petroulia, Manuela Funke, Pascal Zumstein, Sabina Berezowska, Lukas Ebner, Thomas Geiser, Nenad Torbica, Johannes Heverhagen, Alexander Poellinger
OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a progressive lethal chronic lung disease with unclear pathogenesis. Radiological hallmark is the pattern of usual interstitial pneumonia accentuated in peripheral and basal areas with otherwise preserved lung structure. One hypothesis is that alveolar collapse and consequent induration lead to fibrotic transformation of lung tissue. The aim of the study was to investigate normal-appearing tissue during expiration for signs of collapsibility and differences from other diseases or controls...
August 16, 2017: Investigative Radiology
https://www.readbyqxmd.com/read/28800589/prognostic-factors-and-outcomes-in-japanese-lung-transplant-candidates-with-interstitial-lung-disease
#2
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Toyofumi F Chen-Yoshikawa, Takeshi Kubo, Akihiro Aoyama, Hideki Motoyama, Kyoko Hijiya, Shinsaku Tokuda, Yoshinari Nakatsuka, Yuko Yamamoto, Ayako Oshima, Shin-Ichi Harashima, Sonoko Nagai, Toyohiro Hirai, Hiroshi Date, Kazuo Chin
OBJECTIVE: Young patients with advanced interstitial lung disease (ILD) are potential candidates for cadaveric lung transplantation. This study aimed to examine clinical features, outcomes, and prognostic factors in Japanese ILD patients awaiting lung transplantation. METHODS: We investigated the clinical features and outcomes of 77 consecutive candidates with ILD who were referred to Kyoto University Hospital and subsequently actively listed for lung transplant in the Japan Organ Transplant Network between 2010 and 2014...
2017: PloS One
https://www.readbyqxmd.com/read/28796717/utility-of-flexible-bronchoscopic-cryobiopsy-for-diagnosis-of-diffuse-parenchymal-lung-diseases
#3
Robert J Lentz, Trevor M Taylor, Jonathan A Kropski, Kim L Sandler, Joyce E Johnson, Timothy S Blackwell, Fabien Maldonado, Otis B Rickman
BACKGROUND: Initial reports of transbronchial cryobiopsy for diffuse parenchymal lung disease (DPLD) suggest the diagnostic yield approaches that of surgical lung biopsy (SLB) with an excellent safety profile. Centers performing cryobiopsy differ significantly in procedure technique; an optimal technique minimizing complications but still capable of diagnosing a wide range of DPLDs has not been established. We evaluated our practice of flexible bronchoscopic cryobiopsy in a primarily outpatient setting for patients who required a tissue diagnosis for DPLD of uncertain etiology...
August 7, 2017: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/28777013/investigational-drugs-for-idiopathic-pulmonary-fibrosis
#4
Francesco Varone, Giuliano Montemurro, Francesco Macagno, Mariarosaria Calvello, Emanuele Conte, Enrica Intini, Bruno Iovene, Paolo Maria Leone, Pier-Valerio Mari, Luca Richeldi
IPF is a specific form of chronic fibrosing interstitial pneumonia of unknown cause, characterized by progressive worsening in lung function and an unfavorable prognosis. Current concepts on IPF pathogenesis are based on a dysregulated wound healing response, leading to an over production of extracellular matrix. Based on recent research however, several other mechanisms are now proposed as potential targets for novel therapeutic strategies. Areas covered: This review analyzes the current investigational strategies targeting extracellular matrix deposition, tyrosine-kinase antagonism, immune and autoimmune response, and cell-based therapy...
August 10, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28775045/prevalence-and-incidence-of-interstitial-lung-diseases-in-a-multi-ethnic-county-of-greater-paris
#5
Boris Duchemann, Isabella Annesi-Maesano, Camille Jacobe de Naurois, Shreosi Sanyal, Pierre-Yves Brillet, Michel Brauner, Marianne Kambouchner, Sophie Huynh, Jean Marc Naccache, Raphael Borie, Jacques Piquet, Arsène Mekinian, Jerôme Virally, Yurdagul Uzunhan, Jacques Cadranel, Bruno Crestani, Olivier Fain, Francois Lhote, Robin Dhote, Nathalie Saidenberg-Kermanac'h, Paul-André Rosental, Dominique Valeyre, Hilario Nunes
The objective of the study was to estimate the prevalence and incidence of interstitial lung diseases (ILDs) in Seine-Saint-Denis, a multi-ethnic county of Greater Paris, France.Patients with ILDs were identified between January and December 2012 by using several sources; all potentially involved medical specialists from public and private hospitals, community-based pulmonologists and general practitioners, and the Social Security system. Diagnoses were validated centrally by an expert multidisciplinary discussion...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28774636/sleep-as-a-new-target-for-improving-outcomes-in-idiopathic-pulmonary-fibrosis-ipf
#6
REVIEW
Charalampos Mermigkis, Izolde Bouloukaki, Sophia E Schiza
Idiopathic pulmonary fibrosis (IPF) represents the most common type of interstitial pneumonias but remains a disease with a poor outcome. In recent years, two drugs (pirfenidone and nintedanib) have shown promising results at stalling disease progression, however, the interplay of sleep disruption or sleep disorders overall and in relation to medication effectiveness remains understudied. In the past, there was limited interest related to the role of sleep in IPF. Treating physicians tended to point only on the daily disabling symptoms disregarding the possible significant role of sleep alterations or co-existing sleep disorders...
July 31, 2017: Chest
https://www.readbyqxmd.com/read/28770888/spectrum-of-interstitial-lung-disease-from-a-tertiary-care-hospital-in-karachi
#7
Ali Bin Sarwar Zubairi, Maryam Hassan, Talha Shahzad, Sajjad Sarwar, Aamir Abbas, Huzaifa Ahmad, Muhammad Irfan
OBJECTIVE: To determine the clinical features and patterns of interstitial lung disease. METHODS: This retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised record of patients diagnosed with interstitial lung disease from January 2005 to December 2015. All patients aged 16 years and above diagnosed with interstitial lung disease on the basis of clinical features, radiological features on high-resolution computed tomography of the chest, and lung biopsies were included...
July 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28758849/clinical-profile-of-unclassifiable-interstitial-lung-disease-comparison-with-chronic-fibrosing-idiopathic-interstitial-pneumonias
#8
Daniel Traila, Cristian Oancea, Emanuela Tudorache, Ovidiu Fira Mladinescu, Bogdan Timar, Voicu Tudorache
Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28747599/interstitial-pneumonia-with-autoimmune-features
#9
Osamu Nishiyama, Yuji Tohda
  Some patients with idiopathic interstitial pneumonias (IIPs) have clinical features that suggest autoimmune process but not fulfill established criteria for connective tissue disease (CTD). Different terms and criteria have been proposed to describe these patients, which includes undifferentiated connective tissue disease (UCTD), lung dominant connective tissue disease (LD-CTD), and autoimmune-featured interstitial lung disease (AIF-ILD). To marshal these ununiform classification criteria, the "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" proposed a consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity with the term "interstitial pneumonia with autoimmune features" (IPAF)...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28739343/inhibition-of-cell-apoptosis-and-amelioration-of-pulmonary-fibrosis-by-thrombomodulin
#10
Kentaro Fujiwara, Tetsu Kobayashi, Hajime Fujimoto, Hiroki Nakahara, Corina N D'Alessandro-Gabazza, Josephine A Hinneh, Yoshinori Takahashi, Taro Yasuma, Kota Nishihama, Masaaki Toda, Masahiro Kajiki, Yoshiyuki Takei, Osamu Taguchi, Esteban C Gabazza
Pulmonary fibrosis is the terminal stage of a group of idiopathic interstitial pneumonias, of which idiopathic pulmonary fibrosis is the most frequent and fatal form. Recent studies have shown that recombinant human thrombomodulin (rhTM) improves exacerbation and clinical outcome of idiopathic pulmonary fibrosis, but the mechanism remains unknown. This study evaluated the mechanistic pathways of the inhibitory activity of rhTM in pulmonary fibrosis. Transgenic mice overexpressing human transforming growth factor-β1 that develop spontaneously pulmonary fibrosis, and wild-type mice treated with bleomycin were used as models of lung fibrosis...
July 21, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28732823/correlation-of-pulmonary-function-and-usual-interstitial-pneumonia-computed-tomography-patterns-in-idiopathic-pulmonary-fibrosis
#11
Antonella Arcadu, Suzanne C Byrne, Pietro Pirina, Thomas E Hartman, Brian J Bartholmai, Teng Moua
BACKGROUND: Little is known about presenting 'inconsistent' or 'possible' usual interstitial pneumonia (UIP) computed tomography (CT) patterns advancing to 'consistent' UIP as disease progresses in idiopathic pulmonary fibrosis (IPF). We hypothesized that if 'consistent' UIP represented more advanced disease, such a pattern on presentation should also correlate with more severe pulmonary function test (PFT) abnormalities. MATERIAL AND METHODS: Consecutive IPF patients (2005-2013) diagnosed by international criteria with baseline PFT and CT were included...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28690232/-progress-in-sleep-disordered-breathing-in-idiopathic-pulmonary-fibrosis
#12
Ying Zhou, Runxu Song, Danbei Diwu, Yixin Wan
Idiopathic pulmonary fibrosis (IPF) is one of the most common idiopathic interstitial pneumonia. The main symptoms for IPF are dry cough and exertional shortness of breath, which is worsen gradually with the development of pulmonary fibrosis. Patients with IPF often suffer from sleep disordered breathing (SDB). Sleep architecture for these patients including the sleep efficiency, deep sleep, rapid eye movement sleep and arousal index, are changed, which seriously affects the quality of life. In the absence of effective therapies, optimizing the quality of life may become a major therapeutic target for IPF...
June 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28673104/biopsy-in-idiopathic-pulmonary-fibrosis-back-to-the-future
#13
Giulio Rossi, Paolo Spagnolo
Idiopathic Pulmonary Fibrosis (IPF) is a relentlessly progressive, fibrosing interstitial pneumonia characterized by a radiologic and/or histologic pattern of usual interstitial pneumonia (UIP). The availability of two effective anti-fibrotic drugs in IPF has encouraged the identification and treatment of patients in early stages in order to maximize clinical benefit. The ability of high-resolution computed tomography (HRCT) to identify a 'definite' UIP pattern is suboptimal, particularly in the absence of honeycombing...
July 10, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28648751/the-muc5b-promoter-polymorphism-and-telomere-length-in-patients-with-chronic-hypersensitivity-pneumonitis-an-observational-cohort-control-study
#14
Brett Ley, Chad A Newton, Isabel Arnould, Brett M Elicker, Travis S Henry, Eric Vittinghoff, Jeffrey A Golden, Kirk D Jones, Kiran Batra, Jose Torrealba, Christine Kim Garcia, Paul J Wolters
BACKGROUND: Patients with hypersensitivity pneumonitis are at risk of developing pulmonary fibrosis, which is associated with reduced survival. In families with multiple affected members, individuals might be diagnosed as having idiopathic pulmonary fibrosis (IPF) or chronic (fibrotic) hypersensitivity pneumonitis, which suggests these disorders share risk factors. We aimed to test whether the genomic risk factors associated with the development and progression of IPF are also associated with the development of fibrosis and reduced survival in people with chronic hypersensitivity pneumonitis...
August 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28640655/usual-interstitial-pneumonia-can-be-detected-in-transbronchial-biopsies-using-machine-learning
#15
Daniel G Pankratz, Yoonha Choi, Urooj Imtiaz, Grażyna M Fedorowicz, Jessica D Anderson, Thomas V Colby, Jeffrey L Myers, David A Lynch, Kevin K Brown, Kevin R Flaherty, Mark P Steele, Steve D Groshong, Ganesh Raghu, Neil M Barth, P Sean Walsh, Jing Huang, Giulia C Kennedy, Fernando J Martinez
RATIONALE: Usual interstitial pneumonia (UIP) is the histopathologic hallmark of idiopathic pulmonary fibrosis. While UIP can be detected by high-resolution computed tomography (HRCT) of the chest, HRCT results are frequently inconclusive, and pathology from transbronchial biopsy (TBB) has poor sensitivity. Surgical lung biopsy (SLB) may be necessary for a definitive diagnosis. OBJECTIVES: To develop a genomic classifier in tissue obtained by TBB that distinguishes UIP from non-UIP, trained against central pathology as the reference standard...
June 22, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28635201/hamman-rich-syndrome-a-forgotten-entity
#16
William Newmarch, Angelica Puopolo, Madina Weiler, Brian Casserly
The following report outlines the case of a 76-year-old gentleman who presented to the hospital with acute interstitial pneumonitis, a rare and rapidly progressive type of idiopathic interstitial pneumonia. The patient initially presented with a three-week history of progressive shortness of breath and cough which was diagnosed as community acquired pneumonia. Treatment with oral antibiotics was unsuccessful resulting in re-presentation the following week with type one respiratory failure. Investigations revealed widespread inflammatory changes consistent with an acute inflammatory process...
May 18, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
https://www.readbyqxmd.com/read/28626183/desquamative-interstitial-pneumonia-complicated-with-igg4-related-lung-disease
#17
Hideaki Yamakawa, Yoshihiro Suido, Shinko Sadoyama, Yumie Yamanaka, Satoshi Ikeda, Hideya Kitamura, Tomohisa Baba, Koji Okudela, Tamiko Takemura, Takashi Ogura
As an idiopathic interstitial pneumonia, desquamative interstitial pneumonia (DIP) is an uncommon form of interstitial lung disease and is considered to be a smoking- or dust inhalation-related interstitial pneumonia in the majority of cases. However, the details regarding immunoglobulin G4 (IgG4)-related lung disease remain unclear and controversial. We herein report the first case of DIP complicated with IgG4-related lung disease. Even if a patient has a smoking history, we emphasize the importance of exploring the association between DIP and IgG4-related lung disease to clarify the pathogenesis of these two disorders...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28622199/antifibrotic-drugs-as-treatment-of-nonidiopathic-pulmonary-fibrosis-interstitial-pneumonias-the-time-is-now
#18
Michael Kreuter, Julia Wälscher, Jürgen Behr
PURPOSE OF REVIEW: Therapeutic advances in the management of idiopathic pulmonary fibrosis (IPF) has led to improved outcomes with the use of the antifibrotic agents pirfenidone and nintedanib, with a number of randomized studies demonstrating benefits in slowing disease progression in IPF. However, treatment of other fibrosing interstitial lung diseases (ILD) remains challenging. RECENT FINDINGS: Observational and uncontrolled studies investigating pirfenidone and nintedanib in non-IPF ILDs suggest potential benefits, although the data is weak...
September 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28617305/fibrotic-hypersensitivity-pneumonitis-key-issues-in-diagnosis-and-management
#19
REVIEW
Vasileios Kouranos, Joseph Jacob, Andrew Nicholson, Elizabetta Renzoni
The diagnosis of hypersensitivity pneumonitis (HP) relies on the clinical evaluation of a number of features, including a history of significant exposure to potentially causative antigens, physical examination, chest CT scan appearances, bronchoalveolar lavage lymphocytosis, and, in selected cases, histology. The presence of fibrosis is associated with higher morbidity and mortality. Differentiating fibrotic HP from the idiopathic interstitial pneumonias can be a challenge. Furthermore, even in the context of a clear diagnosis of fibrotic HP, the disease behaviour can parallel that of idiopathic pulmonary fibrosis in a subgroup, with inexorable progression despite treatment...
June 15, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28610674/elevated-serum-d-dimer-level-is-associated-with-an-increased-risk-of-acute-exacerbation-in-interstitial-lung-disease
#20
Genta Ishikawa, Samuel O Acquah, Mary Salvatore, Maria L Padilla
BACKGROUND: Early recognition of patients with interstitial lung disease (ILD) who have an increased risk of developing acute exacerbation (AE) or preclinical AE may be clinically useful, since AE is associated with poor outcome and preventive measures would be of interest to ILD researchers. This study evaluated the relationship between elevated serum D-dimer level (≥0.4 mcg/mL) and subsequent AE or preclinical AE in patients with ILD. METHODS: This single-center, retrospective study was performed from October 2009 through September 2015 in patients with ILD who were ≥18 years old and had idiopathic pulmonary fibrosis, other idiopathic interstitial pneumonias, chronic hypersensitivity pneumonitis, ILD related to collagen tissue disease, or combined pulmonary fibrosis/emphysema...
July 2017: Respiratory Medicine
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