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connective tissue disease and lung

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
Joanna E Kusmirek, Maria Daniela Martin, Jeffrey P Kanne
Idiopathic interstitial pneumonias are a heterogeneous group of diffuse lung diseases characterized by distinct clinicopathologic entities with the usual interstitial pneumonia (UIP) being the most common. The pattern of UIP can be seen in idiopathic pulmonary fibrosis (IPF) as well as in secondary causes, most commonly in connective tissue diseases. IPF is usually progressive and associated with a very poor prognosis, and newer therapies pose a risk of serious complications; therefore, diagnostic certainty is crucial...
November 2016: Radiologic Clinics of North America
Jitesh Ahuja, Deepika Arora, Jeffrey P Kanne, Travis S Henry, J David Godwin
Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia...
November 2016: Radiologic Clinics of North America
B Stubbe, S Schipf, C Schäper, S B Felix, A Steveling, M Nauck, H Völzke, H Wallaschofski, N Friedrich, R Ewert, T Ittermann, S Gläser
Background: Diabetes mellitus Type 1 (T1DM) is associated with metabolic and microvascular diseases as part of a multi-organ and multi-systemic disorder. The dense network of capillary vessels in the lungs may change during the course of the development of microangiopathy. The connective tissue as well as alveoli may be subjected to non-enzymatic glycosylation of proteins which may in turn affect pulmonary function. Previous studies investigating lung function in patients with type 1 diabetes have only been performed on small numbers of patients...
October 4, 2016: Experimental and Clinical Endocrinology & Diabetes
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
Sheetu Singh, Bridget F Collins, Bharat B Sharma, Jyotsna M Joshi, Deepak Talwar, Sandeep Katiyar, Nishtha Singh, Lawrence Ho, Jai Kumar Samaria, Parthasarathi Bhattacharya, Rakesh Gupta, Sudhir Chaudhari, Tejraj Singh, Vijay Moond, Sudhakar Pipavath, Jitesh Ahuja, Ravindran Chetambath, Aloke G Ghoshal, Nirmal K Jain, Hj Gayathri Devi, Surya Kant, Parvaiz Koul, Raja Dhar, Rajesh Swarnakar, Suresh Kumar Sharma, Dhrubajyoti J Roy, Kripesh R Sarmah, Bhavin Jankharia, Rodney Schmidt, Santosh K Katiyar, Arpita Jindal, Daya K Mangal, Virendra Singh, Ganesh Raghu
RATIONALE: Interstitial lung disease (ILD) is a heterogeneous group of acute and chronic inflammatory and fibrotic lung diseases. Existing ILD registries have had variable findings. Little is known about the profile of ILDs in India. OBJECTIVES: Create a prospective Registry with validation of diagnoses by multidisciplinary discussion (MDD) to characterize the clinical profile of new onset-ILD in India. METHODS: Prospective recruitment of adult patients with new onset ILD (27 centers in 19 Indian cities, 3/2012-6/2015) with connective tissue disease (CTD) serologies and high-resolution computed tomography (HRCT) chest...
September 29, 2016: American Journal of Respiratory and Critical Care Medicine
Barna Vasarhelyi, Gergely Toldi, Attila Balog
The assessment of the general inflammatory condition of patients with autoimmune connective tissue disorders (ACTD) is a major challenge. The use of traditional inflammatory markers including CRP-levels and erythrocyte sedimentation rate (ESR) is limited by several preanalytical factors and their low specificities. Soluble urokinase plasminogen activator receptor (suPAR) is one of the novel candidate markers that is increasingly used in immune mediated disorders. In our studies we compared suPAR levels of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and ankylosing spondylitis with those of healthy controls...
April 2016: EJIFCC
Akiko Takeuchi, Takashi Matsushita, Kenzo Kaji, Yoshinobu Okamoto, Masahide Yasui, Masayoshi Hirata, Naoto Oishi, Akira Higashi, Mariko Seishima, Tomoya Asano, Manabu Fujimoto, Masataka Kuwana, Kazuhiko Takehara, Yasuhito Hamaguchi
OBJECTIVE: To identify and characterize a novel connective tissue disease (CTD)-related autoantibody (autoAb) directed against scaffold attachment factor B (SAFB). METHODS: AutoAb specificity was analyzed using RNA and protein-immunoprecipitation assays. Autoimmune targets were affinity purified using patients' sera and subjected to liquid chromatography mass spectrometry. RESULTS: By immunoprecipitation assay, 10 sera reacted with a protein with a molecular weight of approximately 160 kDa...
September 24, 2016: Journal of Autoimmunity
Yuh-Feng Lin, I-Jen Chiu, Fong-Yu Cheng, Yu-Hsuan Lee, Ying-Jan Wang, Yung-Ho Hsu, Hui-Wen Chiu
BACKGROUND: Zinc oxide nanoparticles (ZnO NPs) are used in an increasing number of products, including rubber manufacture, cosmetics, pigments, food additives, medicine, chemical fibers and electronics. However, the molecular mechanisms underlying ZnO NP nephrotoxicity remain unclear. In this study, we evaluated the potential toxicity of ZnO NPs in kidney cells in vitro and in vivo. RESULTS: We found that ZnO NPs were apparently engulfed by the HEK-293 human embryonic kidney cells and then induced reactive oxygen species (ROS) generation...
September 27, 2016: Particle and Fibre Toxicology
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
Kamonpun Ussavarungsi, Ryan M Kern, Anja C Roden, Jay H Ryu, Eric S Edell
BACKGROUND: Diagnostic evaluation of patients with diffuse parenchymal lung disease (DPLD) is best achieved by a multidisciplinary team correlating clinical, radiologic, and pathologic features. Surgical lung biopsies remain the gold standard for histopathologic diagnosis of idiopathic interstitial pneumonias. Emerging data suggest an increasing role for transbronchial cryobiopsy (TBC) in DPLD evaluation. We describe our experience with TBC in patients with DPLD. METHODS: We retrospectively reviewed medical records of patients with radiographic features of DPLD who underwent TBC at Mayo Clinic, Rochester, Minnesota in June 2013-September 2015...
September 19, 2016: Chest
Yuko Toyoda, Masaki Hanibuchi, Jun Kishi, Hiroshi Kawano, Shun Morizumi, Seidai Sato, Mayo Kondo, Terumi Takikura, Toshifumi Tezuka, Hisatsugu Goto, Yasuhiko Nishioka
Acute exacerbation (AE) of interstitial lung disease is reported to be developed in not only idiopathic pulmonary fibrosis but also connective tissue disease-associated interstitial pneumonia (CTD-IP). As the significance of AE of CTD-IP has not been so widely recognized, its clinical feature is not fully elucidated. In the present study, we investigated the incidence, clinical features and outcome of AE of CTD-IP. We retrospectively reviewed admitted cases in our department with medical record from 2011 to 2015...
2016: Journal of Medical Investigation: JMI
Koh Uyama, Shoji Sakiyama, Mitsuteru Yoshida, Koichiro Kenzaki, Hiroaki Toba, Yukikiyo Kawakami, Kazumasa Okumura, Hiromitsu Takizawa, Kazuya Kondo, Akira Tangoku
The mortality and morbidity of chronic obstructive pulmonary disease are high. However, no radical therapy has been developed to date. The purpose of this study was to evaluate whether fetal mouse lung tissue can grow and differentiate in the emphysematous lung. Fetal lung tissue from green fluorescent protein C57BL/6 mice at 16 days' gestation was used as donor material. Twelve-month-old pallid mice were used as recipients. Donor lungs were cut into small pieces and implanted into the recipient left lung by performing thoracotomy under anesthesia...
2016: Journal of Medical Investigation: JMI
Jan Hrabeta, Tomas Eckschlager, Marie Stiborova, Zbynek Heger, Sona Krizkova, Vojtech Adam
Zinc ions are essential cofactors of a wide range of enzymes, transcription factors, and other regulatory proteins. Moreover, zinc is also involved in cellular signaling and enzymes inhibition. Zinc dysregulation, deficiency, over-supply, and imbalance in zinc ion transporters regulation are connected with various diseases including cancer. A zinc ion pool is maintained by two types of proteins: (i) zinc-binding proteins, which act as a buffer and intracellular donors of zinc and (ii) zinc transporters responsible for zinc fluxes into/from cells and organelles...
September 16, 2016: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
Brandon T Larsen, Maxwell L Smith, Brett M Elicker, Jessica M Fernandez, Guillermo A Arbo-Oze de Morvil, Carlos A C Pereira, Kevin O Leslie
Context .- Idiopathic pulmonary fibrosis (IPF) is a distinctive clinicopathologic entity and the most common form of progressive diffuse lung scarring in older adults. Idiopathic pulmonary fibrosis manifests histopathologically as the usual interstitial pneumonia pattern. The usual interstitial pneumonia pattern is distinguished by geographically and temporally heterogeneous fibrosis that is peripherally accentuated, often with honeycombing and traction bronchiectasis. Idiopathic pulmonary fibrosis is not the only disease that leads to end-stage lung fibrosis, however, and several other entities may also cause advanced fibrosis...
September 15, 2016: Archives of Pathology & Laboratory Medicine
Florence Morin, Niloufar Kavian, Carole Nicco, Olivier Cerles, Christiane Chéreau, Frédéric Batteux
Systemic sclerosis (SSc) is a connective tissue disorder characterized by fibrosis of the skin and inner organs, vasculopathy, and immunological abnormalities. Recent insights on the implication of STAT3, AKT, and Wnt/β-catenin in fibrosis have prompted us to investigate, in a mouse model of ROS-induced SSc, the effects of niclosamide, an antihelmintic drug that inhibits both of these signaling pathways. SSc was induced in BALB/c mice by daily s.c. injections of hypochlorous acid (HOCl). Mice were treated or not every other day, 5 d a week, for 6 wk, by niclosamide...
September 9, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
Juan Cao, Shike Hou, Hui Ding, Ziquan Liu, Meijuan Song, Xiaojing Qin, Xue Wang, Mengyang Yu, Zhiguang Sun, Jinyang Liu, Shuli Sun, Peixin Xiao, Qi Lv, Haojun Fan
Recently, mesenchymal stem cells (MSCs) are increasingly used as a panacea for multiple types of disease short of effective treatment. Dozens of clinical trials published demonstrated strikingly positive therapeutic effects of MSCs. However, as a specific agent, little research has focused on the dynamic distribution of MSCs after in vivo administration. In this study, we track systemically transplanted allogeneic bone marrow mesenchymal stem cells (BMSCs) in normal rats through bioluminescence imaging (BLI) in real time...
2016: Stem Cells International
B B Singh, R Sharma, J K Sharma, V Mahajan, J P S Gill
Cystic echinococcosis due to Echinococcus granulosus is a serious public health and economic concern in India. The disease is endemic in most of the food producing animals such as cattle, buffalo, sheep, goat and pigs in the country. In this study, tissues comprising of pieces of liver and lungs were collected in 10 % formal buffered saline. The formalin fixed tissues (liver and lungs) from 10 cattle, buffalo, sheep, goat and pigs each were selected and further processed by acetone benzene method for histopathological examinations...
September 2016: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
Natalia Buda, Anna Masiak, Żaneta Smoleńska, Katarzyna Gałecka, Maria Porzezińska, Zbigniew Zdrojewski
Diffuse alveolar haemorrhage (DAH) leads to acute respiratory failure. This is why it requires rapid diagnosis and implementation of appropriate treatment. The standard diagnosis of a patient with DAH includes laboratory tests, diagnostic imagining (X-ray of the chest, high-resolution computed tomography), and bronchoscopy with bronchoalveolar lavage. We present a description of 2 patients diagnosed with systemic connective tissue disease and DAH, monitored with the help of lung ultrasound. To our knowledge, this is the first description of the use of lung ultrasound in DAH in adult patients...
September 2, 2016: Ultrasound Quarterly
Ilia Atanelishvili, Yuichiro Shirai, Tanjina Akter, Atsushi Noguchi, Kurt T Ash, Suniti Misra, Sibnath Ghatak, Richard M Silver, Galina S Bogatkevich
Pulmonary fibrosis represents the terminal stage of a diverse group of lung diseases including scleroderma associated interstitial lung disease. The molecular mechanisms underlying the pathogenesis of lung fibrosis are not well understood and there is a great need for more effective treatment for this lethal disease. We recently discovered a small fragment of hepatocyte growth factor (HGF) receptor MET as a peptide designated "M10," with strong antifibrotic properties. Furthermore, we showed that aspartic acid at position 1398 of MET is essential for M10 generation...
2016: PloS One
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