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lung disease scleroderma

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
Laura Groseanu, Violeta Bojinca, Tania Gudu, Ioana Saulescu, Denisa Predeteanu, Andra Balanescu, Florian Berghea, Daniela Opris, Andreea Borangiu, Cosmin Constantinescu, Magda Negru, Ruxandra Ionescu
OBJECTIVE: Vitamin D has pleiotropic effects including immunomodulatory, cardioprotective, and antifibrotic properties and is thus able to modulate the three main links in scleroderma pathogenesis. The aim of the study was to evaluate the level of vitamin D in patients with systemic sclerosis and to analyze the associations between the concentration of vitamin D and the features of systemic sclerosis. MATERIAL AND METHODS: Fifty-one consecutive patients were evaluated for visceral involvement, immunological profile, activity, severity scores, and quality of life...
June 2016: Eur J Rheumatol
Eliza R Pelrine, Marie-Dominique Ah-Kioon, Meng Zhang, Franck J Barrat, Robert F Spiera, Jessica K Gordon
BACKGROUND: Systemic sclerosis (SSc) is characterized by a wide variety of symptoms and disease manifestations including joint pain, gastrointestinal dysfunction, interstitial lung disease, and cardiomyopathy. QUESTIONS/PURPOSES: Using the Scleroderma Health Assessment Questionnaire (SHAQ) and Short Form-36 (SF-36) we explored how patient-reported physical health, mental health, and functional status related to these clinical characteristics and to cytokine levels utilizing the Hospital for Special Surgery Scleroderma Registry...
October 2016: HSS Journal: the Musculoskeletal Journal of Hospital for Special Surgery
Ya-Ting Chang, Christina K Chan, Inger Eriksson, Pamela Y Johnson, Xiaofang Cao, Christian Westöö, Christian Norvik, Annika Andersson-Sjöland, Gunilla Westergren-Thorsson, Staffan Johansson, Ulf Hedin, Lena Kjellén, Thomas N Wight, Karin Tran-Lundmark
Pulmonary arterial hypertension (PAH) is a lethal condition for which there is no effective curative pharmacotherapy. PAH is characterized by vasoconstriction, wall thickening of pulmonary arteries, and increased vascular resistance. Versican is a chondroitin sulfate proteoglycan in the vascular extracellular matrix that accumulates following vascular injury and promotes smooth-muscle cell proliferation in systemic arteries. Here, we investigated whether versican may play a similar role in PAH. Paraffin-embedded lung sections from patients who underwent lung transplantation to treat PAH were used for immunohistochemistry...
September 2016: Pulmonary Circulation
Kathleen Morrisroe, Molla Huq, Wendy Stevens, Candice Rabusa, Susanna M Proudman, Mandana Nikpour
BACKGROUND: Pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with systemic sclerosis (SSc). We sought to determine the incidence, prevalence and risk factors for PAH development in a large Australian SSc cohort. METHODS: PAH was diagnosed on right heart catheterisation (mPAP >25 and PAWP <15 mmHg at rest). Patients with PH secondary to interstitial lung disease (ILD; defined as abnormal HRCT scan and FVC < 60 %) were excluded...
September 27, 2016: BMC Pulmonary Medicine
S Vemulapalli, L Cohen, V Hsu
OBJECTIVES: Left ventricular diastolic dysfunction (LVDD) is more common in systemic sclerosis (SSc) compared to the general population. Focal myocardial ischaemia and fibrosis may be important in its pathogenesis. LVDD is associated with increased mortality and little is known about the risk factors. Advanced SSc lung complications may accompany LVDD. METHOD: We conducted a cross-sectional study of 300 SSc outpatients with and without LVDD, seen between May 2012 and May 2014, and performed univariate and multivariate regression analyses to determine clinical factors associated with LVDD...
September 16, 2016: Scandinavian Journal of Rheumatology
Gabriele Valentini, Michele Iudici, Ulrich A Walker, Veronika K Jaeger, Murray Baron, Patricia Carreira, László Czirják, Christopher P Denton, Oliver Distler, Eric Hachulla, Ariane L Herrick, Otylia Kowal-Bielecka, Janet Pope, Ulf Müller-Ladner, Gabriela Riemekasten, Jerome Avouac, Marc Frerix, Suzana Jordan, Tünde Minier, Elise Siegert, Voon H Ong, Serena Vettori, Yannick Allanore
BACKGROUND: Validity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised. METHODS: Three investigators assigned an activity score on a 0-10 scale for 97 clinical charts. The median score served as gold standard. Two other investigators labelled the disease as inactive/moderately active or active/very active. Univariate-multivariate linear regression analyses were used to define variables predicting the 'gold standard', their weight and derive an activity index...
September 12, 2016: Annals of the Rheumatic Diseases
Michael D Crowell, Sarah B Umar, W Leroy Griffing, John K DiBaise, Brian E Lacy, Marcelo F Vela
BACKGROUND & AIMS: Systemic scleroderma (SSc) is associated with esophageal aperistalsis and hypotensive esophagogastric junction pressure, although there could be a gradation in esophageal motor dysfunction. We characterized esophageal motor function by high-resolution manometry (HRM), and assessed associations between SSc severity, health-related quality of life (HRQOL), and HRM findings in patients. METHODS: We performed a prospective study of 200 patients with SSc and 102 patients without SSc (controls) who underwent HRM at the Mayo Clinic Arizona Rheumatology Scleroderma Clinic for esophageal manometry, from May 2006 through January 2015...
September 6, 2016: Clinical Gastroenterology and Hepatology
Xueyan Peng, Meagan Moore, Aditi Mathur, Yang Zhou, Huanxing Sun, Ye Gan, Jose D Herazo-Maya, Naftali Kaminski, Xinyuan Hu, Hongyi Pan, Changwan Ryu, Awo Osafo-Addo, Robert J Homer, Carol Feghali-Bostwick, Wassim Fares, Mridu Gulati, Buqu Hu, Chun-Geun Lee, Jack A Elias, Erica L Herzog
Pulmonary fibrosis is a progressive and often fatal condition that is believed to be partially orchestrated by macrophages. Mechanisms that control migration of these cells into and within the lung remain undefined. We evaluated the contributions of the semaphorin receptor, plexin C1 (PLXNC1), and the exocytic calcium sensor, synaptotagmin 7 (Syt7), in these processes. We evaluated the role of PLXNC1 in macrophage migration by using Boyden chambers and scratch tests, characterized its contribution to experimentally induced lung fibrosis in mice, and defined the mechanism for our observations...
September 8, 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
José Mauricio Mota, Luana Guimarães Sousa, Rachel P Riechelmann
Patients with well-differentiated neuroendocrine tumours may develop carcinoid syndrome (CS), which is characterised by flushing, abdominal cramps, diarrhoea, and bronchospasms. In this scenario, long-term secretion of vasoactive substances-serotonin, tachynins, and others, may induce fibrogenic responses in local or distant tissues, leading to complications such as carcinoid heart disease (CHD), mesenteric and/or retroperitoneal fibrosis. Rare cases of lung/pleural fibrosis and scleroderma have also been described...
2016: Ecancermedicalscience
David Launay
No abstract text is available yet for this article.
September 2016: Journal of Rheumatology
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
S Hoa, M Hudson, Y Troyanov, S Proudman, J Walker, W Stevens, M Nikpour, S Assassi, M D Mayes, M Wang, M Baron, M J Fritzler
Autoantibodies directed against the Ku autoantigen are present in systemic sclerosis (SSc) and have been associated with myositis overlap and interstitial lung disease (ILD). However, there is a paucity of data on the clinical correlates of anti-Ku antibodies in the absence of other SSc-specific antibodies. The aim of this study was to assess the clinical correlates of single-specificity anti-Ku in SSc.An international (Canada, Australia, USA, Mexico) cohort of 2140 SSc subjects was formed, demographic and clinical variables were harmonized, and sera were tested for anti-Ku using a line immunoassay...
August 2016: Medicine (Baltimore)
Norihiro Nagamura, Seikon Kin
Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC...
August 2016: Nagoya Journal of Medical Science
Elizabeth R Volkmann, Donald P Tashkin
Although interstitial lung disease accounts for the majority of deaths in patients with systemic sclerosis, treatment options for this manifestation of systemic sclerosis are limited. Few high quality, randomized controlled trials exist for systemic sclerosis-related interstitial lung disease, and historically, studies have favored the use of cyclophosphamide. However, the benefit of cyclophosphamide for systemic sclerosis-related interstitial lung disease is tempered by its complex adverse event profile. More recent studies have demonstrated the effectiveness of mycophenolate for systemic sclerosis-related interstitial lung disease, including the recently published Scleroderma Lung Study II...
August 25, 2016: Annals of the American Thoracic Society
Cody M Lee, Diana Girnita, Arundhati Sharma, Surabhi Khanna, Jean M Elwing
Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia...
2016: Case Reports in Rheumatology
N Del Papa, F Onida, E Zaccara, G Saporiti, W Maglione, E Tagliaferri, R Andracco, D Vincenti, T Montemurro, L Mircoli, C Vitali, A Cortelezzi
We retrospectively evaluated the efficacy of autologous hematopoietic stem cell transplantation (AHSCT) in 18 patients with rapidly progressive diffuse cutaneous systemic sclerosis (rp-dcSSc), and compared their disease outcomes with those of 36 demographically- and clinically-matched patients treated with conventional therapies. Cutaneous involvement, by performing modified Rodnan skin score (mRss), lung diffusion capacity, by measuring diffusing capacity of lung for carbon monoxide (DLCO), and disease activity, by applying the European Scleroderma Study Group (ESSG) scoring system, were the outcome variables measured at the baseline time and then every 12 months for the following 60 months in both the AHSCT-treated patients and the control group...
August 22, 2016: Bone Marrow Transplantation
Pim Kranenburg, Wieneke M T van den Hombergh, Hanneke K A Knaapen-Hans, Frank H J van den Hoogen, Jaap Fransen, Madelon C Vonk
OBJECTIVE: LcSSc is associated with ACAs and a mild course, whereas dcSSc is associated with anti-topoisomerase antibodies (ATAs) and a more severe course. However, ATAs are also present in lcSSc. Little is known about survival and organ involvement in this subgroup. The aim of this study is to determine whether survival and organ involvement of lcSSc ATA-positive patients differs from lcSSc ATA-negative or dcSSc ATA-positive patients. Furthermore, transition from lcSSc to dcSSc was evaluated...
August 12, 2016: Rheumatology
Manabu Hayama, Shingo Sato, Takayuki Shiroyama, Takuji Nishida, Takashi Nishihara, Norio Okamoto
A 68-year-old woman with interstitial lung disease related to dermatomyositis and systemic scleroderma was admitted to our hospital with fever and dyspnoea. Although the fever was reduced after antibiotic therapy, a left pneumothorax suddenly occurred on day 27 after admission. A continuous air leak persisted despite chest drainage with three tubes and repeated pleurodesis. Chest computed tomography (CT) images showed a cavitary lesion with a pinhole in the left upper division, which was suspected to be the affected lesion with the air leak...
July 2016: Respirology Case Reports
Donald P Tashkin, Michael D Roth, Philip J Clements, Daniel E Furst, Dinesh Khanna, Eric C Kleerup, Jonathan Goldin, Edgar Arriola, Elizabeth R Volkmann, Suzanne Kafaja, Richard Silver, Virginia Steen, Charlie Strange, Robert Wise, Fredrick Wigley, Maureen Mayes, David J Riley, Sabiha Hussain, Shervin Assassi, Vivien M Hsu, Bela Patel, Kristine Phillips, Fernando Martinez, Jeffrey Golden, M Kari Connolly, John Varga, Jane Dematte, Monique E Hinchcliff, Aryeh Fischer, Jeffrey Swigris, Richard Meehan, Arthur Theodore, Robert Simms, Suncica Volkov, Dean E Schraufnagel, Mary Beth Scholand, Tracy Frech, Jerry A Molitor, Kristin Highland, Charles A Read, Marvin J Fritzler, Grace Hyun J Kim, Chi-Hong Tseng, Robert M Elashoff
BACKGROUND: 12 months of oral cyclophosphamide has been shown to alter the progression of scleroderma-related interstitial lung disease when compared with placebo. However, toxicity was a concern and without continued treatment the efficacy disappeared by 24 months. We hypothesised that a 2 year course of mycophenolate mofetil would be safer, better tolerated, and produce longer lasting improvements than cyclophosphamide. METHODS: This randomised, double-blind, parallel group trial enrolled patients from 14 US medical centres with scleroderma-related interstitial lung disease meeting defined dyspnoea, pulmonary function, and high-resolution CT (HRCT) criteria...
September 2016: Lancet Respiratory Medicine
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