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interstitial lung disease on connective tissue disease

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https://www.readbyqxmd.com/read/28704913/treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-the-pulmonologist-s-point-of-view
#1
REVIEW
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
https://www.readbyqxmd.com/read/28666014/radiologic-pleuroparenchymal-fibroelastosis-like-lesion-in-connective-tissue-disease-related-interstitial-lung-disease
#2
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
https://www.readbyqxmd.com/read/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#3
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
https://www.readbyqxmd.com/read/28471413/role-of-autoantibodies-in-the-diagnosis-of-connective-tissue-disease-ild-ctd-ild-and-interstitial-pneumonia-with-autoimmune-features-ipaf
#4
REVIEW
Adelle S Jee, Stephen Adelstein, Jane Bleasel, Gregory J Keir, MaiAnh Nguyen, Joanne Sahhar, Peter Youssef, Tamera J Corte
The diagnosis of interstitial lung disease (ILD) requires meticulous evaluation for an underlying connective tissue disease (CTD), with major implications for prognosis and management. CTD associated ILD (CTD-ILD) occurs most commonly in the context of an established CTD, but can be the first and/or only manifestation of an occult CTD or occur in patients who have features suggestive of an autoimmune process, but not meeting diagnostic criteria for a defined CTD-recently defined as "interstitial pneumonia with autoimmune features" (IPAF)...
May 4, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28467213/interstitial-pneumonia-with-autoimmune-features-value-of-histopathology
#5
Ayodeji Adegunsoye, Justin M Oldham, Eleanor Valenzi, Cathryn Lee, Leah J Witt, Lena Chen, Steven Montner, Jonathan H Chung, Imre Noth, Rekha Vij, Mary E Strek, Aliya N Husain
CONTEXT: - Patients with idiopathic interstitial pneumonia may display evidence of autoimmunity without meeting criteria for a defined connective tissue disease. A recent European Respiratory Society/American Thoracic Society statement proposed research criteria for interstitial pneumonia with autoimmune features (IPAF), which includes findings from the clinical, serologic, and morphologic domains. OBJECTIVES: - To investigate the importance of histopathologic criteria within the morphologic domain and to report our methodology for identifying these features...
July 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28350485/ct-findings-radiologic-pathologic-correlation-and-imaging-predictors-of-survival-for-patients-with-interstitial-pneumonia-with-autoimmune-features
#6
Jonathan H Chung, Steven M Montner, Ayodeji Adegunsoye, Cathryn Lee, Justin M Oldham, Aliya N Husain, Heber MacMahon, Imre Noth, Rekha Vij, Mary E Strek
OBJECTIVE: The objective of this study is to determine the CT findings and patterns of interstitial pneumonia with autoimmune features (IPAF) and to assess whether imaging can predict survival for patients with IPAF. MATERIALS AND METHODS: The study included 136 subjects who met the criteria for IPAF and had diagnostic-quality chest CT scans obtained from 2006 to 2015; a total of 74 of these subjects had pathologic samples available for review within 1 year of chest CT examination...
March 28, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28344982/transbronchial-cryobiopsy-in-interstitial-lung-disease-experience-in-106-cases-how-to-do-it
#7
Antonio Bango-Álvarez, Miguel Ariza-Prota, Hector Torres-Rivas, Luis Fernández-Fernández, Amador Prieto, Inmaculada Sánchez, Maria Gil, Ana Pando-Sandoval
Transbronchial biopsy using forceps (TBB) is the first diagnostic technique performed on patients with interstitial lung disease (ILD). However, the small size of the samples and the presence of artefacts in the tissue obtained make the yield variable. Our objectives were 1) to attempt to reproduce transbronchial cryobiopsy under the same conditions with which we performed conventional TBB, that is, in the bronchoscopy unit without intubating the patient and without fluoroscopy or general anaesthesia; 2) to describe the method used for its execution; and 3) to analyse the diagnostic yield and its complications...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28323056/fty720-promotes-pulmonary-fibrosis-when-administered-during-the-remodelling-phase-following-a-bleomycin-induced-lung-injury
#8
David R Gendron, Anne-Marie Lemay, Pascale Blais Lecours, Valérie Perreault-Vallières, Carole-Ann Huppé, Ynuk Bossé, Marie-Renée Blanchet, Geneviève Dion, David Marsolais
Fibrosis complicates numerous pathologies including interstitial lung diseases. Sphingosine analogs such as FTY720 can alleviate lung injury-induced fibrosis in murine models. Contradictorily, FTY720 also promotes in vitro processes normally leading to fibrosis and high doses in vivo foster lung fibrosis by enhancing vascular leakage into the lung. The goal of this study was to determine the effect of low doses of FTY720 on lung fibrosis triggered by an acute injury in mice. We first defined the time-boundaries delimiting the inflammatory and remodelling phases of an injury elicited by bleomycin based on neutrophil counts, total lung capacity and lung stiffness...
June 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28297158/unclassifiable-interstitial-lung-diseases-clinical-characteristics-and-survival
#9
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/28279287/a-42-year-old-woman-with-anemia-shock-and-ischemic-stroke-after-lung-transplantation
#10
Abhishek Biswas, Ali Ataya, Juan C Salgado, Satish Chandrasekharan, Tiago N Machuca, Amir M Emtiazjoo
A 42-year-old woman with mixed connective tissue disease-associated interstitial lung disease underwent bilateral lung transplantation. She had an uneventful surgery and was extubated 3 h later. Induction immunosuppression therapy included methylprednisolone 500 mg intraoperatively, basiliximab (anti-IL-2 monoclonal antibody) on days 0 and 4 after transplantation, and methylprednisolone 125 mg intravenously bid for 2 days following surgery. Maintenance immunosuppression therapy consisted of prednisone 20 mg daily, mycophenolate mofetil 750 mg bid, and enteral tacrolimus 0...
March 2017: Chest
https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#11
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
https://www.readbyqxmd.com/read/28274531/an-analysis-of-the-clinical-features-of-lung-cancer-in-patients-with-connective-tissue-diseases
#12
Atsuro Saijo, Masaki Hanibuchi, Hisatsugu Goto, Yuko Toyoda, Toshifumi Tezuka, Yasuhiko Nishioka
BACKGROUND: Patients with connective tissue diseases (CTDs) are at increased risk for lung cancer (LC); interstitial lung disease (ILD) is a common form of organ dysfunction in cases of CTD. However, the influence of ILD on the treatment and prognosis in LC patients with CTD is unclear. METHODS: Between January 2010 and December 2014, 27 patients among all patients with CTD at our institution were diagnosed with primary LC. We retrospectively analyzed the clinical features, treatment modalities, and outcomes of these patients, and evaluated the potential prognostic factors...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28137497/interstitial-pneumonia-with-autoimmune-features-clinical-radiologic-and-histological-characteristics-and-outcome-in-a-series-of-57-patients
#13
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/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#14
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
June 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28063071/interstitial-lung-disease-in-systemic-sclerosis-current-and-future-treatment
#15
REVIEW
Roberto Giacomelli, Vasiliki Liakouli, Onorina Berardicurti, Piero Ruscitti, Paola Di Benedetto, Francesco Carubbi, Giuliana Guggino, Salvatore Di Bartolomeo, Francesco Ciccia, Giovanni Triolo, Paola Cipriani
Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc-ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients...
June 2017: Rheumatology International
https://www.readbyqxmd.com/read/28005084/-comorbidity-diseases-in-adults-with-diagnosed-interstitial-lung-diseases-among-inhabitants-of-the-silesian-voivodeship-poland
#16
Ewa Niewiadomska, Małgorzata Kowalska, Jan E Zejda
BACKGROUND: Interstitial lung diseases form a group of chronic diseases associated with a significant worsening of the quality of life. Proper management of these diseases involves the recognition and treatment of comorbidities, so it implies high direct and indirect costs of therapy. The lack of epidemiological data on the total incidence of interstitial diseases in Poland, as well as of information on their increasing incidence in other European countries justify investigations into epidemiological situation in the Silesian voivodeship (the southern region of Poland)...
December 22, 2016: Medycyna Pracy
https://www.readbyqxmd.com/read/27977621/lung-cancer-development-in-patients-with-connective-tissue-disease-related-interstitial-lung-disease-a-retrospective-observational-study
#17
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)
https://www.readbyqxmd.com/read/27686729/molecular-mechanisms-underlying-fibrosis-and-elastin-destruction-in-childhood-interstitial-lung-diseases
#18
Enas A Hamed, Mostafa M El-Saied, Khaled Saad, Hazem Abu-Zeid Yousef, Amany O Mohamed, Dina Sabry
OBJECTIVE: This study aimed to evaluate fibrosis and elastin destruction in childhood interstitial lung disease (chILD) patients. METHODS: Sixty patients and twenty healthy children were recruited. On admission, evaluation of chILD severity was made using Fan chILD score. Participants provided urine and blood samples. Plasma levels of transforming growth factor (TGF)-β1, connective tissue growth factor (CCN2), soluble factor related apoptosis (sFas) and long non-coding RNAs and urinary levels of desmosine/urinary creatinine (UDes/UCr) were measured...
September 21, 2016: Pathophysiology: the Official Journal of the International Society for Pathophysiology
https://www.readbyqxmd.com/read/27664227/cardiac-tamponade-as-a-manifestation-of-severe-dermatomyositis
#19
Benjamin James Kurth, Vanya Wagler, Michael Keith
In connective tissue disorders, the incidence of pericardial disease and pericardial effusion can be up to 58%, and if untreated, it can lead to cardiac tamponade which can be fatal. Physicians must have a high index of suspicion for this disease as diagnosis can be delayed while evaluating more common causes of tachycardia and hypotension in the immunosuppressed (ie, sepsis). We present a 55-year-old woman with a severe case of dermatomyositis, marked by significant weight loss, a bedridden state and hallmark cutaneous findings...
2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27564852/clinical-features-of-idiopathic-interstitial-pneumonia-with-systemic-sclerosis-related-autoantibody-in-comparison-with-interstitial-pneumonia-with-systemic-sclerosis
#20
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Shinichiro Iso, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Patients with idiopathic interstitial pneumonias sometimes have a few features of connective tissue disease (CTD) and yet do not fulfil the diagnostic criteria for any specific CTD. OBJECTIVE: This study was conducted to elucidate the characteristics, prognosis, and disease behavior in patients with interstitial lung disease (ILD) associated with systemic sclerosis (SSc)-related autoantibodies. METHODS: We retrospectively analyzed medical records of 72 ILD patients: 40 patients with SSc (SSc-ILD) and 32 patients with SSc-related autoantibody-positive ILD but not with CTD (ScAb-ILD), indicating lung-dominant CTD with SSc-related autoantibody...
2016: PloS One
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