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Idiopathic interstitial pneumonias

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https://www.readbyqxmd.com/read/28324200/prevalence-and-characterization-of-non-sicca-onset-primary-sj%C3%A3-gren-syndrome-with-interstitial-lung-involvement
#1
Manfredi Andreina, Sebastiani Marco, Cerri Stefania, Cassone Giulia, Bellini Pietrantonio, Della Casa Giovanni, Luppi Fabrizio, Ferri Clodoveo
Primary Sjögren syndrome (pSS)-related interstitial lung disease (ILD) involved about 10-20% of patients. In 20% of cases, ILD can be diagnosed before pSS; anyway, few studies have investigated the frequency of ILD as the first clinically relevant manifestation of pSS, generally referred to retrospective studies. Aim of our prospective study was to describe prevalence, clinical, serological, and instrumental features of non-sicca onset pSS patients with interstitial lung involvement. During a period of 48 months, all consecutive patients diagnosed as pSS were enrolled...
March 21, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28300574/sex-steroid-receptor-expression-in-idiopathic-pulmonary-fibrosis
#2
Mitra Mehrad, Humberto E Trejo Bittar, Samuel A Yousem
Usual interstitial pneumonia (UIP) is characterized by progressive scarring of the lungs and is associated with high morbidity and mortality despite therapeutic interventions. Sex steroid receptors have been demonstrated to play an important role in chronic lung conditions; however, their significance is unknown in patients with UIP. We retrospectively reviewed 40 idiopathic UIP cases for the expression of hormonal receptors. Forty cases including 10 normal lung, 10 cryptogenic organizing pneumonia (COP), 10 idiopathic organizing diffuse alveolar damage (DAD), 7 hypersensitivity pneumonitis (HP) and 3 nonspecific interstitial pneumonitis (NSIP) served as controls...
March 11, 2017: Human Pathology
https://www.readbyqxmd.com/read/28300570/idiopathic-interstitial-pneumonia-associated-with-autoantibodies-a-large-case-series-followed-over-one-year
#3
Bridget F Collins, Charles F Spiekerman, Megan A Shaw, Lawrence A Ho, Jennifer Hayes, Carolyn A Spada, Caroline M Stamato, Ganesh Raghu
BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia (IIP), particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF) or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: Retrospective single center study; ANOVA analyses compared differences in mean change in forced vital capacity (FVC) and diffusion capacity (DLCO) over 1-year among 124 well defined patients (20 AI-ILD [positive autoantibodies with or without symptoms of CTD], 15 IPAF, 36 CTD-ILD, 53 "Lone-IPF" [patients with IPF without any autoantibodies]) RESULTS: 75% of patients with AI-ILD, 33% IPAF, and 33% CTD-ILD had UIP...
March 11, 2017: Chest
https://www.readbyqxmd.com/read/28297158/unclassifiable-interstitial-lung-diseases-clinical-characteristics-and-survival
#4
Charlotte Hyldgaard, Elisabeth Bendstrup, Athol U Wells, Ole Hilberg
BACKGROUND AND OBJECTIVE: Unclassifiable disease in chronic interstitial lung disease (ILD) is a common and challenging problem but has been insufficiently studied. The 2013 update of the international multidisciplinary classification of the idiopathic interstitial pneumonias presented a classification based on observed disease behaviour with the purpose of providing guidance to clinicians in the management of these patients. The aim of this study was to apply the new disease behaviour classification (DBC) and the previously validated ILD-gender age physiology (GAP) score to a cohort of unclassifiable ILD patients and to assess the prognostic value of these two composite approaches...
April 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28292522/guidelines-for-the-medical-treatment-of-idiopathic-pulmonary-fibrosis
#5
Antoni Xaubet, María Molina-Molina, Orlando Acosta, Elena Bollo, Diego Castillo, Estrella Fernández-Fabrellas, José Antonio Rodríguez-Portal, Claudia Valenzuela, Julio Ancochea
Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis...
March 11, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28260097/pathway%C3%A2-based-detection-of-idiopathic-pulmonary-fibrosis-at-an-early-stage
#6
Guojun Zhou, Fangxia Zhang, Yufang Liu, Bin Sun
Idiopathic pulmonary fibrosis (IPF) is the most common interstitial pneumonia and the most aggressive interstitial lung disease. Usually, IPF is confirmed by the histopathological pattern of typical interstitial pneumonia and requires an integrated multidisciplinary approach from pulmonologists, radiologists and pathologists. However, these diagnoses are performed at an advanced stage of IPF. At present, pathway‑based detection requires investigation, as it can be performed at an early stage of the disease...
March 1, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28247107/effect-of-collagen-vascular-disease-associated-interstitial-lung-disease-on-the-outcomes-of-lung-cancer-surgery
#7
Hideyuki Maeda, Masato Kanzaki, Kei Sakamoto, Tamami Isaka, Kunihiro Oyama, Masahide Murasugi, Takamasa Onuki
PURPOSE: This study compared the effect of collagen vascular disease-associated interstitial lung disease (CVD-ILD) with that of idiopathic interstitial pneumonias (IIPs) on the outcomes of lung cancer surgery. METHODS: This study retrospectively reviewed the medical records of patients who underwent surgery for non-small cell lung cancer (NSCLC) and compared the data of 16 patients with CVD-ILD with those of 70 patients with IIPs. The patterns of interstitial lung disease (ILD) on chest computed tomography were classified into usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) patterns...
February 28, 2017: Surgery Today
https://www.readbyqxmd.com/read/28247041/volatile-organic-compounds-in-exhaled-breath-of-idiopathic-pulmonary-fibrosis-for-discrimination-from-healthy-subjects
#8
Yu-Ichi Yamada, Gen Yamada, Mitsuo Otsuka, Hirotaka Nishikiori, Kimiyuki Ikeda, Yasuaki Umeda, Hirofumi Ohnishi, Koji Kuronuma, Hirofumi Chiba, Jörg Ingo Baumbach, Hiroki Takahashi
Purpose Human breath analysis is proposed with increasing frequency as a useful tool in clinical application. We performed this study to find the characteristic volatile organic compounds (VOCs) in the exhaled breath of patients with idiopathic pulmonary fibrosis (IPF) for discrimination from healthy subjects. Methods VOCs in the exhaled breath of 40 IPF patients and 55 healthy controls were measured using a multi-capillary column and ion mobility spectrometer. The patients were examined by pulmonary function tests, blood gas analysis, and serum biomarkers of interstitial pneumonia...
March 1, 2017: Lung
https://www.readbyqxmd.com/read/28245857/the-diagnostic-value-of-the-bronchoalveolar-lavage-in-interstitial-lung-diseases
#9
Boubacar Efared, G Ebang-Atsame, Sani Rabiou, Abdoulsalam S Diarra, Layla Tahiri, Nawal Hammas, Mohamed Smahi, Bouchra Amara, Mohamed C Benjelloun, Mounia Serraj, Laila Chbani, Hinde El Fatemi
OBJECTIVE: Bronchoalveolar lavage (BAL) is a diagnostic tool often used during the management of interstitial lung diseases (ILD). However, its diagnostic value in discrimination between entities comprising the very heterogenous group of ILD, is still a controversial issue. The objective of our study is to assess the diagnostic value of BAL in the management of ILD, by comparing the cytological findings in BAL fluid among the different diseases of this group. METHODS: It was a retrospective, observational study of 151 patients between January 2012 and December 2015...
March 1, 2017: Journal of Negative Results in Biomedicine
https://www.readbyqxmd.com/read/28230086/classification-of-idiopathic-interstitial-pneumonias-using-anti-myxovirus-resistance-protein-1-autoantibody
#10
Yoshimasa Hamano, Hiroshi Kida, Shoichi Ihara, Akihiro Murakami, Masahiro Yanagawa, Ken Ueda, Osamu Honda, Lokesh P Tripathi, Toru Arai, Masaki Hirose, Toshimitsu Hamasaki, Yukihiro Yano, Tetsuya Kimura, Yasuhiro Kato, Hyota Takamatsu, Tomoyuki Otsuka, Toshiyuki Minami, Haruhiko Hirata, Koji Inoue, Izumi Nagatomo, Yoshito Takeda, Masahide Mori, Hiroyoshi Nishikawa, Kenji Mizuguchi, Takashi Kijima, Masanori Kitaichi, Noriyuki Tomiyama, Yoshikazu Inoue, Atsushi Kumanogoh
Chronic fibrosing idiopathic interstitial pneumonia (IIP) can be divided into two main types: idiopathic pulmonary fibrosis (IPF), a steroid-resistant and progressive disease with a median survival of 2-3 years, and idiopathic non-specific interstitial pneumonia (INSIP), a steroid-sensitive and non-progressive autoimmune disease. Although the clinical courses of these two diseases differ, they may be difficult to distinguish at diagnosis. We performed a comprehensive analysis of serum autoantibodies from patients definitively diagnosed with IPF, INSIP, autoimmune pulmonary alveolar proteinosis, and sarcoidosis...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28202030/upregulation-of-interleukin-33-and-thymic-stromal-lymphopoietin-levels-in-the-lungs-of-idiopathic-pulmonary-fibrosis
#11
Jong-Uk Lee, Hun Soo Chang, Hyeon Ju Lee, Chang An Jung, Da Jeong Bae, Hyun Ji Song, Jong Sook Park, Soo-Taek Uh, Young Hoon Kim, Ki-Hyun Seo, Choon-Sik Park
BACKGROUND: Innate T helper type 2 (Th2) immune responses mediated by interleukin (IL)-33, thymic stromal lymphopoietin (TSLP), and IL-25 have been shown to play an important role in pulmonary fibrosis of animal models; however, their clinical implications remain poorly understood. METHODS: TSLP, IL-25, and IL-33 concentrations were measured in bronchoalveolar lavage fluids obtained from normal controls (NCs; n = 40) and from patients with idiopathic pulmonary fibrosis (IPF; n = 100), non-specific interstitial pneumonia (NSIP; n = 22), hypersensitivity pneumonitis (HP; n = 20), and sarcoidosis (n = 19)...
February 15, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28198728/personalized-medicine-in-interstitial-lung-diseases
#12
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...
February 13, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28182861/diffuse-pulmonary-ossification-in-fibrosing-interstitial-lung-diseases-prevalence-and-associations
#13
Ryoko Egashira, Joseph Jacob, Maria A Kokosi, Anne-Laure Brun, Alexandra Rice, Andrew G Nicholson, Athol U Wells, David M Hansell
Purpose To investigate the prevalence of diffuse pulmonary ossification (DPO) in patients with fibrosing interstitial lung disease (ILD) and determine whether there are differences among the types of ILDs. Materials and Methods Institutional review board approval was given and patient consent was not required for this study. The study population comprised 892 consecutive patients with fibrosing ILD, including 456 patients with idiopathic pulmonary fibrosis (IPF) (men, 366; women, 90; median age, 72 years [range, 38-93 years]), 244 with nonspecific interstitial pneumonia (men, 79; women, 165; median age, 60...
February 9, 2017: Radiology
https://www.readbyqxmd.com/read/28149568/recommendations-for-the-management-of-idiopathic-pulmonary-fibrosis-in-south-africa-a-position-statement-of-the-south-african-thoracic-society
#14
REVIEW
Coenraad F N Koegelenberg, Gillian M Ainslie, Keertan Dheda, Brian W Allwood, Michelle L Wong, Umesh G Lalloo, Mohamed S Abdool-Gaffar, Hoosain Khalfey, Elvis M Irusen
Idiopathic pulmonary fibrosis (IPF) is a very specific form of a chronic, progressive fibroproliferative interstitial pneumonia of unknown aetiology. The disease is generally associated with a poor prognosis. Several international evidence-based guidelines on the diagnosis and management of IPF and other interstitial lung diseases (ILDs) have been published and updated in the last decade, and while the body of evidence for the use of some treatment modalities has grown, others have been shown to be futile and even harmful to patients...
December 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28137498/evaluation-of-urinary-desmosines-as-a-noninvasive-diagnostic-biomarker-in-patients-with-idiopathic-pleuroparenchymal-fibroelastosis-ppfe
#15
Yoshiyuki Oyama, Noriyuki Enomoto, Yuzo Suzuki, Masato Kono, Tomoyuki Fujisawa, Naoki Inui, Yutaro Nakamura, Shigeki Kuroishi, Koshi Yokomura, Mikio Toyoshima, Shiro Imokawa, Keiji Oishi, Satoshi Watanabe, Kazuo Kasahara, Tomohisa Baba, Takashi Ogura, Hiroshi Ishii, Kentaro Watanabe, Yasuhiko Nishioka, Takafumi Suda
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial pneumonia with upper lobe predominance and fibroelastosis. Although definite diagnosis requires surgical lung biopsy (SLB), SLB is often difficult because of its complications such as refractory pneumothorax. OBJECTIVE: To evaluate urinary desmosines (degradation product of mature elastin) as a novel biomarker in patients with PPFE. METHODS: Biopsy-proven patients with PPFE (n = 14) were prospectively enrolled...
February 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28137497/interstitial-pneumonia-with-autoimmune-features-clinical-radiologic-and-histological-characteristics-and-outcome-in-a-series-of-57-patients
#16
Kais Ahmad, Thomas Barba, Delphine Gamondes, Marylise Ginoux, Chahera Khouatra, Paolo Spagnolo, Mary Strek, Françoise Thivolet-Béjui, Julie Traclet, Vincent Cottin
BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) has recently been defined by an international Taskforce to characterize interstitial lung disease associated with systemic manifestations limited to subtle serological and clinical autoimmune abnormalities and not fulfilling the international criteria for the diagnosis of a given connective tissue disease. OBJECTIVE: to report on a series of patients with IPAF, and to compare their outcome to that of a cohort of patients with idiopathic pulmonary fibrosis (IPF)...
February 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28137485/fam13a-polymorphism-as-a-prognostic-factor-in-patients-with-idiopathic-pulmonary-fibrosis
#17
Chihiro Hirano, Shinichiro Ohshimo, Yasushi Horimasu, Hiroshi Iwamoto, Kazunori Fujitaka, Hironobu Hamada, Noboru Hattori, Nobuaki Shime, Francesco Bonella, Josune Guzman, Ulrich Costabel, Nobuoki Kohno
BACKGROUND: Family with sequence similarity 13, member A (FAM13A) variants have been associated with susceptibility to chronic lung diseases. A recent genome-wide association study has shown an association between a polymorphism in FAM13A rs2609255 and idiopathic interstitial pneumonias in a Caucasian population. However, the relationship between rs2609255 polymorphism and prognosis in idiopathic interstitial pneumonias has not been investigated. METHODS: Sixty-five patients with idiopathic pulmonary fibrosis (IPF) and 310 Japanese healthy volunteers were enrolled in this study...
February 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28125151/pleuroparenchymal-fibroelastosis-report-of-two-cases-in-brazil
#18
Paula Silva Gomes, Christina Shiang, Gilberto Szarf, Ester Nei Aparecida Martins Coletta, Carlos Alberto de Castro Pereira
Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe...
January 23, 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28122457/idiopathic-pulmonary-fibrosis-current-status-recent-progress-and-emerging-targets
#19
Yi-Min Liu, Kunal Nepali, Jing-Ping Liou
Idiopathic pulmonary fibrosis (IPF), a chronic and progressive fibrosing interstitial pneumonia, is a fatal lung disease with a median survival time of 3-5 years. Problems in accurate diagnosis, poor prognosis, limited clinical therapy, and high mortality rate together demonstrate that the development of efficient therapeutic strategies for IPF is an important future endeavor. Deeper understanding of pathogenesis and identification of biomarkers and pathways involved might lead in the future to the emergence of some agents as novel therapeutics for IPF...
January 26, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28120410/restrictive-allograft-syndrome-and-idiopathic-pleuroparenchymal-fibroelastosis-do-they-really-have-the-same-histology
#20
M Angeles Montero, Tina Osadolor, Reena Khiroya, M Teresa Salcedo, Jan Lukas Robertus, Alexandra Rice, Andrew G Nicholson, Antonio Roman, Victor Monforte
AIMS: Restrictive Allograft Syndrome (RAS) and idiopathic pleuroparenchymal fibroelastosis (IPPFE) are two different diseases reported to share the same histology. RAS relates to chronic allograft dysfunction in lung transplantation with IPPFE being a rare condition in native lungs. Our aim is to compare their histologies, alongside biopsies of usual interstitial pneumonia (UIP), to determine if there are differences that might help to elucidate the pathogenesis. METHODS AND RESULTS: We selected 4 post-mortem allograft lungs from patients who developed clear clinical RAS pattern, 5 biopsies diagnosed as IPPFE, 5 UIP biopsies and 5 sections of normal lung...
January 25, 2017: Histopathology
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