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interstitial lung disease associated vascular collagen disease

Jürgen Behr, Petra Neuser, Antje Prasse, Michael Kreuter, Klaus Rabe, Carmen Schade-Brittinger, Jasmin Wagner, Andreas Günther
BACKGROUND: Pirfenidone is currently approved in the EU for the treatment of mild to moderate idiopathic pulmonary fibrosis (IPF) and offers a beneficial risk-benefit profile. However, there are several other, progressive fibrotic lung diseases, in which conventional anti-inflammatory therapy is not sufficiently effective and antifibrotic therapies may offer a novel treatment option. METHODS/DESIGN: We designed a study protocol for inclusion of patients with progressive fibrotic lung disease despite conventional anti-inflammatory therapy (EudraCT 2014-000861-32)...
September 6, 2017: BMC Pulmonary Medicine
So-My Koo, Soo-Taek Uh
Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). This may be due to the lack of CTD cases available, the difficulty of histological confirmation of ILD, and the various types of CTD and ILD...
July 2017: Korean Journal of Internal Medicine
Haruhiko Furusawa, Makiko Sugiura, Chieko Mitaka, Naohiko Inase
BACKGROUND AND OBJECTIVE: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is recognized as an important cause of mortality. AE has also been reported in patients with other interstitial lung diseases such as idiopathic non-specific interstitial pneumonia (NSIP) and interstitial pneumonia associated with collagen vascular disease (CVD). Current therapies such as high-dose corticosteroid with immunosuppressive agents have provided little benefit for AE. Direct hemoperfusion (DHP) with a polymyxin B-immobilized fibre column (PMX) was originally developed for the treatment of endotoxaemia...
April 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
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...
September 2017: Surgery Today
Tetsuro Sawata, Masashi Bando, Masayuki Nakayama, Naoko Mato, Hideaki Yamasawa, Yukihiko Sugiyama
Objective The influence of smoking on the pathogenesis and clinical course of interstitial pneumonia has recently attracted attention. To clarify the influence of smoking on the clinical patient characteristics and therapeutic effects in patients with interstitial pneumonia presenting with a non-specific interstitial pneumonia (NSIP) pattern, we compared the clinical patient characteristics and therapeutic effects in smokers and nonsmokers in this study. Methods We divided 31 NSIP (16 idiopathic nonspecific interstitial pneumonia and 15 collagen vascular disease-associated nonspecific interstitial pneumonia) patients into smoker and non-smoker groups for each case...
2016: Internal Medicine
M N A Vogel, M Kreuter, H-U Kauczor, C-P Heußel
CLINICAL/METHODICAL ISSUE: Pulmonary complications are frequent in patients with collagen vascular diseases (CVD). Frequent causes are a direct manifestation of the underlying disease, side effects of specific medications and lung infections. STANDARD RADIOLOGICAL METHODS: The standard radiological procedure for the work-up of pulmonary pathologies in patients with CVD is multidetector computed tomography (MDCT) with thin-slice high-resolution reconstruction. PERFORMANCE: The accuracy of thin-slice CT for the identification of particular disease patterns is very high...
October 2016: Der Radiologe
Nobuyuki Ashizawa, Shigeki Nakamura, Shotaro Ide, Masato Tashiro, Takahiro Takazono, Yoshifumi Imamura, Taiga Miyazaki, Koichi Izumikawa, Yoshihiro Yamamoto, Katsunori Yanagihara, Yoshitsugu Miyazaki, Shigeru Kohno
A 76-year-old woman received long-term immunosuppressive treatment for collagen vascular disease-associated interstitial pneumonia. The patient developed a cavitary mass lesion in the right lower lung field, and both nontuberculous mycobacteria and Aspergillus spp. were isolated after bronchial washing. The patient underwent a right lower lobectomy but developed Aspergillus empyema. Empyema due to Aspergillus spp. is a rare and life-threatening condition; however, the standard therapeutic strategies for treating Aspergillus empyema are not clear...
2016: Internal Medicine
Osamu Nishiyama, Ryo Yamazaki, Hiroyuki Sano, Takashi Iwanaga, Yuji Higashimoto, Hiroaki Kume, Yuji Tohda
Background. Six-minute walk test (6MWT) has 3 measurement outcomes, which are walk distance, desaturation, and symptom. The aim of this study was to examine whether routinely measured right-heart catheter (RHC) data correlate with 6MWT outcomes in patients with interstitial lung disease (ILD). Methods. Between June 2010 and December 2012, consecutive patients with ILD who underwent evaluation, including pulmonary function test, hemodynamic studies with right-heart catheter, and 6MWT as routine general practice, were recruited...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
Nour R Dayeh, Jonathan Ledoux, Jocelyn Dupuis
Left heart diseases (LHD) represent the most prevalent cause of pulmonary hypertension (PH), yet there are still no approved therapies that selectively target the pulmonary circulation in LHD. The increase in pulmonary capillary pressure due to LHD is a triggering event leading to physical and biological alterations of the pulmonary circulation. Acutely, mechanosensitive endothelial dysfunction and increased capillary permeability combined with reduced fluid resorption lead to the development of interstitial and alveolar oedema...
July 2016: Progress in Cardiovascular Diseases
Dominique Israël-Biet, Jean Pastré, Karine Juvin
Bonchoalveolar lavage is a safe and poorly invasive tool with a great diagnostic value particularly in diffuse infiltrative pulmonary diseases (IPD). In specific instances, it allows for a definite diagnosis (alveolar hemorrhage; alveolar proteinosis, lipidoses, infiltrative malignant diseases, opportunistic infections), obviating the need to perform more invasive diagnostic procedures like video-assisted surgical biopsy. In inflammatory IPD, either idiopathic, diagnostic or associated with inhaled antigens or with collagen vascular diseases for instance, it represents a crucial orientation diagnostic tool, considerably narrowing the spectrum of potential differential diagnosis...
December 2015: La Revue du Praticien
Tsukasa Okamoto, Mayumi Fujii, Haruhiko Furusawa, Kimitake Tsuchiya, Yasunari Miyazaki, Naohiko Inase
BACKGROUND: It is believed that Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D) are useful biomarkers for the diagnosis of various types of interstitial lung diseases, including hypersensitivity pneumonitis (HP). The clinical features of chronic HP are similar to those of idiopathic interstitial pneumonias, especially idiopathic pulmonary fibrosis (IPF). OBJECTIVE: We sought to clarify the usefulness of serum KL-6 and SP-D for the diagnosis and management of chronic HP...
December 2015: Respiratory Medicine
Toshiharu Tsutsui, Yasunari Miyazaki, Tsukasa Okamoto, Tomoya Tateishi, Haruhiko Furusawa, Kimitake Tsuchiya, Toshihide Fujie, Meiyo Tamaoka, Hiroyuki Sakashita, Yuki Sumi, Naohiko Inase
BACKGROUND: Chronic hypersensitivity pneumonitis (HP) is induced by the inhalation of specific antigens. Patients with chronic HP may be able to improve their prognosis by avoiding these antigens. Chronic HP is often difficult to distinguish from idiopathic interstitial pneumonias (IIPs). OBJECTIVE: This study was performed to find out how antigen avoidance tests contribute to the diagnosis of chronic HP. METHODS: A retrospective analysis was conducted on 265 patients who underwent 2-week antigen avoidance tests between April 2002 and March 2012...
September 2015: Respiratory Investigation
Hiroshi Furukawa, Shomi Oka, Kota Shimada, Kiyoe Masuo, Fumiaki Nakajima, Shunichi Funano, Yuki Tanaka, Akiko Komiya, Naoshi Fukui, Tatsuya Sawasaki, Kenji Tadokoro, Masato Nose, Naoyuki Tsuchiya, Shigeto Tohma
Interstitial lung disease (ILD) is frequently associated with collagen disease. It is then designated as collagen vascular disease-associated ILD (CVD-ILD), and influences patients' prognosis. The prognosis of acute-onset diffuse ILD (AoDILD) occurring in patients with collagen disease is quite poor. Here, we report our investigation of auto-antibody (Ab) profiles to determine whether they may be useful in diagnosing CVD-ILD or AoDILD in collagen disease. Auto-Ab profiles were analyzed using the Lambda Array Beads Multi-Analyte System, granulocyte immunofluorescence test, Proto-Array Human Protein Microarray, AlphaScreen assay, and glutathione S-transferase capture enzyme-linked immunosorbent assay in 34 patients with rheumatoid arthritis (RA) with or without CVD-ILD and in 15 patients with collagen disease with AoDILD...
2015: Biomarker Insights
Peter S Hegan, Anthony A Lanahan, Michael Simons, Mark S Mooseker
In mice and humans, loss of myosin VI (Myo6) function results in deafness, and certain Myo6 mutations also result in cardiomyopathies in humans. The current studies have utilized the Snell's waltzer (sv) mouse (a functional null mutation for Myo6) to determine if this mouse also exhibits cardiac defects and thus used to determine the cellular and molecular basis for Myo6-associated heart disease. Myo6 is expressed in mouse heart where it is predominantly expressed in vascular endothelial cells (VECs) based on co-localization with the VEC cell marker CD31...
August 2015: Cytoskeleton
Amjad Al-Rajhi, Elisa Ferreira Brega, Neil C Colman
Silica (silicon dioxide) occupational exposure has been linked to both pulmonary and extra-pulmonary toxicity. Silicosis is the major pulmonary toxicity, which has also been associated with the development of collagen-vascular disease and with anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis, especially perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA). The most common pulmonary manifestations of microscopic polyangitis (MPA) are interstitial fibrosis and alveolar hemorrhage. We describe a patient who had unusual presentation of microscopic polyangitis, characterized by lung hemorrhage, rapidly progressive glomerulonephritis, pleuropericarditis and pulmonary embolism that was associated with a history of silica exposure and radiologic evidence for silicosis...
2015: Respiratory Medicine Case Reports
Yohannes T Ghebremariam, John P Cooke, William Gerhart, Carol Griego, Jeremy B Brower, Melanie Doyle-Eisele, Benjamin C Moeller, Qingtao Zhou, Lawrence Ho, Joao de Andrade, Ganesh Raghu, Leif Peterson, Andreana Rivera, Glenn D Rosen
BACKGROUND: The beneficial outcome associated with the use of proton pump inhibitors (PPIs) in idiopathic pulmonary fibrosis (IPF) has been reported in retrospective studies. To date, no prospective study has been conducted to confirm these outcomes. In addition, the potential mechanism by which PPIs improve measures of lung function and/or transplant-free survival in IPF has not been elucidated. METHODS: Here, we used biochemical, cell biological and preclinical studies to evaluate regulation of markers associated with inflammation and fibrosis...
August 1, 2015: Journal of Translational Medicine
Hiroshi Furukawa, Shomi Oka, Kota Shimada, Naoyuki Tsuchiya, Shigeto Tohma
Interstitial lung disease (ILD) is a chronic, progressive fibrotic lung disease with a dismal prognosis. ILD of unknown etiology is referred to as idiopathic interstitial pneumonia (IIP), which is sporadic in the majority of cases. ILD is frequently accompanied by rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), and other autoimmune diseases, and is referred to as collagen vascular disease-associated ILD (CVD-ILD). Susceptibility to ILD is influenced by genetic and environmental factors...
2015: Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine
Lilian Tiemi Kuranishi, Kevin O Leslie, Rimarcs Gomes Ferreira, Ester Aparecida Ney Coletta, Karin Mueller Storrer, Maria Raquel Soares, Carlos Alberto de Castro Pereira
BACKGROUND: Airway-centered Interstitial Fibrosis (ACIF) is a common pathologic pattern observed in our practice. OBJECTIVES: The objectives of this study are to describe the causes associated with ACIF in a large sample of patients and its effect on survival. METHODS: A retrospective study in three centers of interstitial lung disease in São Paulo, between January of 1995 and December of 2012. The surgical lung biopsy specimens were reviewed by three pathologists...
2015: Respiratory Research
Shota Nakashima, Tomoyuki Kakugawa, Hirokazu Yura, Masaomi Tomonaga, Tatsuhiko Harada, Atsuko Hara, Shintaro Hara, Masayuki Nakano, Eiki Yamasaki, Noriho Sakamoto, Yuji Ishimatsu, Hajime Isomoto, Bernadette R Gochuico, Anthony F Suffredini, Hiroshi Mukae, Hisao Kurazono, Toshiya Hirayama, Joel Moss, Shigeru Kohno
OBJECTIVE: Prior reports suggested that infection with Helicobacter pylori was associated with respiratory diseases; pathogenetic mechanisms however, were not defined. We tested the hypothesis that VacA, an exotoxin of H. pylori, a gastric pathogen, was aspirated into the lung and could stimulate secretion of inflammatory cytokines by lung epithelial cells. METHODS: The presence of VacA was determined by immunohistochemistry in surgical lung biopsy tissue samples from 72 patients with interstitial pneumonia...
May 8, 2015: Biochemical and Biophysical Research Communications
Junji Itai, Shinichiro Ohshimo, Yoshiko Kida, Kohei Ota, Yasumasa Iwasaki, Nobuyuki Hirohashi, Francesco Bonella, Josune Guzman, Ulrich Costabel, Nobuoki Kohno, Koichi Tanigawa
BACKGROUND: Direct hemoperfusion with polymyxin B-immobilized fiber (PMX-DHP) might be beneficial for treating acute exacerbation (AE) of interstitial pneumonia (IP). Venovenous extracorporeal membranous oxygenation (VV-ECMO) is an emerging tool to avoid ventilator-induced lung injury. This is a report presenting the first three patients with AE of IP treated with a combined therapy of PMX-DHP and VV-ECMO. CASE PRESENTATION: Patient 1 was a 68-year-old male with acute interstitial pneumonia, patient 2 a 67-year-old male with AE of idiopathic pulmonary fibrosis, and patient 3 a 61-year-old female with AE of collagen vascular disease-associated interstitial pneumonia...
2014: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
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