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Connective tissue disease related ILD

Sahajal Dhooria, Ritesh Agarwal, Inderpaul Singh Sehgal, Kuruswamy Thurai Prasad, Mandeep Garg, Amanjit Bal, Ashutosh Nath Aggarwal, Digambar Behera
BACKGROUND: The spectrum of interstitial lung diseases (ILDs) have mainly been reported from the developed countries; data from developing countries is sparse and conflicting. The aim of this study is to describe the distribution of various ILDs from a developing country. METHODS: This is an analysis of prospectively collected clinical, radiological and histological data of consecutive subjects (age >12 years) with ILDs from a single tertiary care medical center...
2018: PloS One
Whitney Gannon, David J Lederer, Mauer Biscotti, Azka Javaid, Nina Patel, Daniel Brodie, Matthew Bacchetta, Matthew Baldwin
BACKGROUND: We aimed to examine short- and long-term mortality in a mixed population of interstitial lung disease (ILD) patients with acute respiratory failure, and to identify those at lower versus higher risk of in-hospital death. METHODS: We conducted a single-center retrospective cohort study of 126 consecutive adults with ILD admitted to an intensive care unit (ICU) for respiratory failure at a tertiary care hospital between 2010 and 2014 who did not undergo lung transplantation during their hospitalization...
January 15, 2018: Chest
Hayley Barnes, Anne E Holland, Glen P Westall, Nicole Sl Goh, Ian N Glaspole
BACKGROUND: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death...
January 3, 2018: Cochrane Database of Systematic Reviews
Tomohiko Takahashi, Satoru Hayata, Akihiro Kobayashi, Yuna Onaka, Takeshi Ebihara, Terufumi Hara
BACKGROUND AND OBJECTIVE: Pulmonary arterial hypertension (PAH) is an intractable and rare disease and the accumulation of clinical evidence under real-world setting is needed. A post-marketing surveillance for the endothelin receptor antagonist ambrisentan (Volibris tablet) has been conducted by all-case investigation since September 2010. This paper is an interim report on the safety and efficacy of ambrisentan in 702 patients with PAH. METHODS: PAH patients aged 15 years or older were subjected to the analysis...
December 27, 2017: Clinical Drug Investigation
Anupam Kumar, Sujith V Cherian, Robert Vassallo, Eunhee S Yi, Jay H Ryu
Tobacco exposure results in various changes to the airways and lung parenchyma. Although emphysema represents the more common injury pattern, in some individuals, cigarette smoke injures alveolar epithelial cells and other lung cells, resulting in diffuse infiltrates and parenchymal fibrosis. Smoking can trigger interstitial injury patterns mediated via recruitment and inappropriate persistence of myeloid and other immune cells, including eosinophils. As our understanding of the role of cigarette smoke constituents in triggering lung injury continues to evolve, so does our recognition of the spectrum of smoking-related interstitial lung changes...
December 5, 2017: Chest
Philippa Ashmore, Mohammed Tikly, Michelle Wong, Claudia Ickinger
To investigate the frequency, severity and predictors of interstitial lung disease (ILD) in a cohort of South Africans with systemic sclerosis (SSc). Retrospective record review of SSc patients attending a tertiary Connective Tissue Diseases Clinic. Patients with ILD, defined by a combination of clinical findings, imaging, and lung function tests were compared to patients without ILD in terms of demographics, clinical features and autoantibodies. The majority (86.8%) of the 151 patients included were of Black ethnicity, 40% had ILD, of whom 39% had moderate-severe lung disease...
November 28, 2017: Rheumatology International
Koji Habe, Hideo Wada, Ayaka Higashiyama, Tomoko Akeda, Kenshiro Tsuda, Ryoko Mori, Masato Kakeda, Takeshi Matsumoto, Kohshi Ohishi, Keiichi Yamanaka, Naoyuki Katayama, Hitoshi Mizutani
This study aimed to examine the hemostatic abnormalities in patients with systemic sclerosis (SSc) and the relationship between these abnormalities and thrombotic events (THEs), focusing on the difference in diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The plasma levels of ADAMTS-13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13), von Willebrand factor (VWF), VWF propeptide (VWFpp), d-dimer, and soluble fibrin (SF) were measured in 233 patients with SSc. The relationship between their levels and organ involvement, including THEs and interstitial lung disease (ILD), was evaluated...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
Hailan Li, Zeng Xiong, Jinkang Liu, Yisha Li, Bin Zhou
To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed.
 Results: The classifications for 127 patients were as follows: 36 cases of rheumatoid arthritis (28...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Mohammad Alsumrain, Federica De Giacomi, Shireen Mirza, Teng Moua
BACKGROUND: Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. We assessed the frequency of incident CTD-ILD as detected by autoimmune serology testing and presenting clinical signs and symptoms. METHODS: Consecutive patients seen at our institution over a four year period with newly diagnosed uncharacterized ILD and autoimmune serologic testing were included...
August 23, 2017: Respiratory Research
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
Yasunori Enomoto, Yutaro Nakamura, Thomas V Colby, Takeshi Johkoh, Hiromitsu Sumikawa, Koji Nishimoto, Katsuhiro Yoshimura, Sayomi Matsushima, Yoshiyuki Oyama, Hironao Hozumi, Masato Kono, Tomoyuki Fujisawa, Noriyuki Enomoto, Naoki Inui, Toshihide Iwashita, Takafumi Suda
BACKGROUND: Radiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD). MATERIALS AND METHODS: A total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, which was defined as bilateral, upper lobe, and subpleural dense consolidations with or without pleural thickening on chest high-resolution computed tomography...
2017: PloS One
Peter Saunders, Vicky Tsipouri, Gregory J Keir, Deborah Ashby, Marcus D Flather, Helen Parfrey, Daphne Babalis, Elisabetta A Renzoni, Christopher P Denton, Athol U Wells, Toby M Maher
BACKGROUND: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little...
June 15, 2017: Trials
Atsushi Suzuki, Yasuhiro Kondoh, Aryeh Fischer
Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD). Although the majority of patients with CTD-ILD are stable or slowly progressive, a significant group exhibits a more severe and progressive decline. Interstitial pneumonia with autoimmune features (IPAF) describes the subset of patients with interstitial pneumonia who have features suggesting underlying autoimmunity, but whose features fall short of a clear diagnosis of CTD. Areas covered: In this focused review, we discuss recent advances in early detection, prognostic evaluation, and management of autoimmune forms of ILD...
July 2017: Expert Review of Respiratory Medicine
Si-Jie Yuan, Hai-Ting Xie, Zhong-Li Li
OBJECTIVE: To investigate the clinical significance of plasma levels of hypersensitive C-reactive protein (hs-CRP), fibriogen and D-dimmer (D-DI) in patients with connective tissue disease (CTD)-related interstitial lung disease (CTD-ILD). METHODS: Sixty-nine patients with interstitial lung disease admitted in Zhujiang Hospital between January, 2010 and April, 2016, including 29 with CTD-ILD and 40 with non-CTD-ILD were analyzed for plasma levels of hs-CRP, fibriogen and D-DI, with 25 healthy subjects as the control group...
March 20, 2017: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
Leona M Dowman, Christine F McDonald, Catherine J Hill, Annemarie L Lee, Kathryn Barker, Claire Boote, Ian Glaspole, Nicole S L Goh, Anne M Southcott, Angela T Burge, Rebecca Gillies, Alicia Martin, Anne E Holland
BACKGROUND: Uncertainty exists regarding the clinical relevance of exercise training across the range of interstitial lung diseases (ILDs). OBJECTIVE: To establish the impact of exercise training in patients with ILDs of differing aetiology and severity. METHODS: 142 participants with ILD (61 idiopathic pulmonary fibrosis (IPF), 22 asbestosis, 23 connective tissue disease-related ILD (CTD-ILD) and 36 with other aetiologies) were randomised to either 8 weeks of supervised exercise training or usual care...
July 2017: Thorax
María Laura Alberti, Francisco Paulin, Heidegger Mateos Toledo, Martín Eduardo Fernández, Fabián Matías Caro, Jorge Rojas-Serrano, Mayra Edith Mejía
OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns)...
December 12, 2016: Reumatología Clinica
Yasunori Enomoto, Naoki Inui, Katsuhiro Yoshimura, Koji Nishimoto, Kazutaka Mori, Masato Kono, Tomoyuki Fujisawa, Noriyuki Enomoto, Yutaro Nakamura, Toshihide Iwashita, Takafumi Suda
Previous studies have reported that patients with idiopathic pulmonary fibrosis occasionally develop lung cancer (LC). However, in connective tissue disease (CTD)-related interstitial lung disease (ILD), there are few data regarding the LC development. The aim of the present study was to evaluate the clinical significance of LC development in patients with CTD-ILD. A retrospective review of our database of 562 patients with ILD between 2000 and 2014 identified 127 patients diagnosed with CTD-ILD. The overall and cumulative incidences of LC were calculated...
December 2016: Medicine (Baltimore)
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Anne Laure Brun, Ryoko Egashira, Ronald Karwoski, Maria Kokosi, Athol U Wells, David M Hansell
BACKGROUND: To evaluate computer-based computer tomography (CT) analysis (CALIPER) against visual CT scoring and pulmonary function tests (PFTs) when predicting mortality in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). To identify outcome differences between distinct CTD-ILD groups derived following automated stratification of CALIPER variables. METHODS: A total of 203 consecutive patients with assorted CTD-ILDs had CT parenchymal patterns evaluated by CALIPER and visual CT scoring: honeycombing, reticular pattern, ground glass opacities, pulmonary vessel volume, emphysema, and traction bronchiectasis...
November 23, 2016: BMC Medicine
Gemma Lepri, Jerome Avouac, Paolo Airò, Francisco Anguita Santos, Silvia Bellando-Randone, Jelena Blagojevic, Francisco Garcia Hernàndez, Jose Antonio Gonzalez Nieto, Serena Guiducci, Suzana Jordan, Vidya Limaye, Britta Maurer, Albert Selva-O'Callaghan, Valeria Riccieri, Oliver Distler, Marco Matucci-Cerinic, Yannick Allanore
OBJECTIVES: Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy. METHODS: A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23)...
September 2016: Clinical and Experimental Rheumatology
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