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lung disease scleroderma

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https://www.readbyqxmd.com/read/29425538/-etiological-profile-of-secondary-raynaud-s-phenomenon-in-an-internal-medicine-department-about-121-patients
#1
T Ben Salem, M Tougorti, S Bziouech, M Lamloum, M Khanfir, I Ben Ghorbel, M H Houman
INTRODUCTION: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. METHODS: A descriptive retrospective study including patients with secondary Raynaud's phenomenon followed in a tertiary internal medicine department between 2000 and 2013...
February 2018: Journal de Médecine Vasculaire
https://www.readbyqxmd.com/read/29394994/long-term-survival-in-bilateral-lung-transplantation-for-scleroderma-related-lung-disease
#2
Edward Y Chan, Ahmad Goodarzi, Neeraj Sinha, Duc T Nguyen, J Georges Youssef, Erik E Suarez, Thomas Kaleekal, Edward A Graviss, Brian A Bruckner, Thomas E MacGillivray, Scott A Scheinin
BACKGROUND: Lung disease is the leading cause of morbidity and death in scleroderma patients, but scleroderma is often considered a contraindication to lung transplantation because of concerns for worse outcomes. We evaluated whether 5-year survival in scleroderma patients after lung transplantation differed from other patients with restrictive lung disease. METHODS: This was a single-center, retrospective cohort study of all patients undergoing bilateral lung transplantation for scleroderma-related pulmonary disease between January 2006 and December 2014...
January 27, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29382307/emphysematous-change-with-scleroderma-associated-interstitial-lung-disease-the-potential-contribution-of-vasculopathy
#3
Hideaki Yamakawa, Tamiko Takemura, Tae Iwasawa, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Hideya Kitamura, Tomohisa Baba, Shinichiro Iso, Koji Okudela, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers but also in never-smokers. This study aimed to describe a new finding characterized by peculiar emphysematous change with SSc-associated ILD (SSc-ILD). METHODS: We conducted a retrospective review of 21 consecutive patients with SSc-ILD diagnosed by surgical lung biopsy and focused on the radio-pathological correlation of the emphysematous change...
January 30, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29369808/bicaudal-d2-is-a-novel-autoantibody-target-in-systemic-sclerosis-that-shares-a-key-epitope-with-cenp-a-but-has-a-distinct-clinical-phenotype
#4
REVIEW
Marvin J Fritzler, Marie Hudson, May Y Choi, Michael Mahler, Mianbo Wang, Chelsea Bentow, Jay Milo, Murray Baron
We studied the clinical correlations and epitopes of autoantibodies directed to a novel autoantigen, Bicaudal D (BICD2), in systemic sclerosis (SSc) and reviewed its relationship to centromere protein A (CENP-A). 451 SSc sera were tested for anti-BICD2 using a paramagnetic bead immunoassay and then univariate and multivariate logistic regression was used to study the association between anti-BICD2 and demographic and clinical parameters as well as other SSc-related autoantibodies. Epitope mapping was performed on solid phase matrices...
January 21, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29322341/interstitial-lung-disease-in-systemic-sclerosis-data-from-the-spanish-scleroderma-study-group
#5
D Sánchez-Cano, N Ortego-Centeno, J L Callejas, V Fonollosa Plá, R Ríos-Fernández, C Tolosa-Vilella, G Espinosa-Garriga, D Colunga-Argüelles, M V Egurbide-Arberas, M Rubio-Rivas, M Freire, J J Ríos-Blanco, L Trapiella-Martínez, M Rodríguez-Carballeira, A Marín-Ballvé, X Pla-Salas, C P Simeón-Aznar
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group...
January 10, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29316366/impact-of-radiation-therapy-on-scleroderma-and-cancer-outcomes-in-scleroderma-patients-with-breast-cancer
#6
Dhaval J Shah, Ram Hirpara, Corrie L Poelman, Adrianne Woods, Laura K Hummers, Fredrick M Wigley, Jean L Wright, Arti Parekh, Virginia D Steen, Robyn T Domsic, Ami A Shah
OBJECTIVES: We examined SSc patients with breast cancer to 1) identify the prevalence of radiation complications and 2) examine SSc outcomes in SSc patients who received radiation therapy (RT) as part of their cancer treatment. METHODS: Patients with SSc and breast cancer were identified from the Johns Hopkins and University of Pittsburgh Scleroderma Center databases. We examined whether erythema, blistering, ulceration, or thickening of the skin developed in the RT port...
January 5, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29297205/cyclophosphamide-for-connective-tissue-disease-associated-interstitial-lung-disease
#7
REVIEW
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
https://www.readbyqxmd.com/read/29246248/factors-associated-with-the-6-minute-walk-distance-in-patients-with-systemic-sclerosis
#8
Sébastien Sanges, Jonathan Giovannelli, Vincent Sobanski, Sandrine Morell-Dubois, Hélène Maillard, Marc Lambert, Céline Podevin, Nicolas Lamblin, Pascal De Groote, Jean-François Bervar, Thierry Perez, Régis Matran, Martine Rémy-Jardin, Pierre-Yves Hatron, Éric Hachulla, David Launay
BACKGROUND: There is an ongoing debate regarding the relevance of the 6-minute walking distance (6MWD) in systemic sclerosis (SSc) assessment, widely used as a usual test in these patients as well as an outcome measure in clinical trials. In this work, we aimed to assess the associations between the 6MWD and various disease parameters in patients with SSc. METHODS: Consecutive patients followed in our SSc National Reference Centre were included in this cross-sectional study if they fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism criteria for SSc...
December 15, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29241641/the-systemic-sclerosis-questionnaire-sysq-validation-of-the-translation-of-the-original-german-version-into-spanish-and-its-relationship-to-the-disease-and-to-quality-of-life
#9
Maria Pilar Cruz-Domínguez, Moisés Casarrubias-Ramírez, Victor Gasca-Martínez, Cindy Maldonado García, Rosa Angélica Carranza-Muleiro, Gabriela Medina, Grettel García-Collinot, Daniel Hector Montes-Cortes
OBJECTIVE: Translation, transculturation and validity of the self-administered questionnaire for functionality (Systemic Sclerosis Questionnaires [SySQ]) for use in Spanish patients with systemic sclerosis and its relationship to the severity of the disease and to quality of life. PATIENTS AND METHODS: We conducted an observational analytical study to perform a cross-cultural validation of the self-administered questionnaire on functionality in scleroderma. The validity of the form and content was evaluated by an expert panel...
December 11, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/29214548/first-clinical-symptom-as-a-prognostic-factor-in-systemic-sclerosis-results-of-a-retrospective-nationwide-cohort-study
#10
Manuel Rubio-Rivas, Xavier Corbella, Melany Pestaña-Fernández, Carles Tolosa-Vilella, Alfredo Guillen-Del Castillo, Dolores Colunga-Argüelles, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Pablo Segovia-Alonso, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar, E Callejas Moraga, E Calvo, C Carbonell, M J Castillo, A J Chamorro, D Colunga, X Corbella, M V Egurbide, G Espinosa, V Fonollosa, M Freire, F J García Hernández, R González León, A Guillén Del Castillo, N Iniesta, R Lorenzo, A B Madroñero, B Marí, A Marín, N Ortego-Centeno, M Pérez Conesa, M Pestaña, X Pla, J J Ríos Blanco, M Rodríguez Carballeira, M Rubio Rivas, M Ruiz Muñoz, L Sáez Comet, P Segovia, C P Simeón, A Soto, E Tarí, J A Todolí, C Tolosa, L Trapiella, J A Vargas Hitos, G Verdejo
The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited...
December 7, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29207307/systemic-sclerosis-clinical-manifestations-anesthetic-and-orthopedic-considerations-in-a-patient
#11
Obada Hasan, Muneeba Jessar, Muhammad Ashar, Shahryar Noordin, Tashfeen Ahmad
INTRODUCTION: Systemic sclerosis is a rare and progressive multisystem autoimmune disorder that is characterized pathologically by vascular abnormalities, connective tissue sclerosis and atrophy of skin and various internal organs (e.g., alimentary tract, lungs, heart, kidney, CNS), and autoantibodies. With an unknown etiology, Scleroderma is a complex polygenetic disease. A recent Genome Wide Association Study (GWAS) confirmed a strong association with the Major Histocompatibility Complex (MHC) and autoimmunity...
December 2, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29164837/serum-surfactant-protein-d-and-exhaled-nitric-oxide-as-biomarkers-of-early-lung-damage-in-systemic-sclerosis
#12
Alida Benfante, Riccardo Messina, Alessandra Paternò, Nicola Scichilone
BACKGROUND: Interstitial lung disease (ILD) complicates the course of systemic sclerosis (SSc), representing the main cause of death in these patients. The identification of parameters that can predict the early onset and progression of ILD in SSc represents an unmet need in clinical practice. The study was designed to explore whether the surfactant proteins (SP) A and D may be used as noninvasive tools for the early identification of ILD in SSc. Alveolar exhaled nitric oxide (NO) was investigated as a surrogate marker of distal inflammation...
November 21, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29151520/dropped-head-syndrome-and-the-presence-of-rimmed-vacuoles-in-a-muscle-biopsy-in-scleroderma-polymyositis-overlap-syndrome-associated-with-anti-ku-antibody
#13
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29142074/stat3-controls-col1a2-enhancer-activation-cooperatively-with-junb-regulates-type-i-collagen-synthesis-post-transcriptionally-and-is-essential-for-lung-myofibroblast-differentiation
#14
Ioannis Papaioannou, Shiwen Xu, Christopher P Denton, David J Abraham, Markella Ponticos
Fibroblast differentiation is key cellular process that underlies the process of fibrosis, a deadly complication of fibrotic diseases like Scleroderma (SSc). This transition coincides with the overproduction of Collagen type I (COL1) and other extracellular matrix proteins. High level expression of the collagen type 1α2 subunit (COL1A2), requires the engagement of a far upstream enhancer, whose activation is strongly dependent on the AP1 factor JunB. We now report that STAT3 also binds the COL1A2 enhancer and is essential for RNA polymerase recruitment, without affecting JunB binding...
November 15, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29135399/-investigation-of-parameters-related-to-prognosis-in-diffuse-parenchymal-lung-diseases-prognosis-in-interstitial-lung-diseases
#15
Coşkun Canıvar, Züleyha Bingöl, Zeki Kılıçaslan, Tülin Çağatay, N Gülfer Okumuş
Introduction: Parameters related to prognosis in diffuse parenchymal lung disease (DPLD) have a decisive influence on treatment and follow-up processes. We aimed to define baseline characteristics and factors that effect the mortality of the group of patients with DPLD and to determine distinctions between subgroups. Materials and Methods: Demographic characteristics, complaints, comorbidity, treatment, pulmonary function tests, echocardiographic findings, six minute walking test (6MWT), arterial blood gases analysis, radiological findings and survival time were collected from outpatient clinics database...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29104809/mycobacterium-intracellulare-infection-mimicking-progression-of-scleroderma
#16
Simon Krabbe, Merete Engelhart, Sören Thybo, Søren Jacobsen
This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29093152/pomalidomide-in-patients-with-interstitial-lung-disease-due-to-systemic-sclerosis-a-phase-ii-multicenter-randomized-double-blind-placebo-controlled-parallel-group-study
#17
Vivien M Hsu, Christopher P Denton, Robyn T Domsic, Daniel E Furst, Maureen Rischmueller, Marina Stanislav, Virginia D Steen, Jörg H W Distler, Shimon Korish, Alyse Cooper, Suktae Choi, Peter H Schafer, Gerald Horan, Douglas R Hough
OBJECTIVE: To evaluate the safety and efficacy of pomalidomide (POM) on forced vital capacity (FVC), modified Rodnan skin score (mRSS), and gastrointestinal (GI) symptomatology over 52 weeks of treatment in patients with interstitial lung disease due to systemic sclerosis (SSc). METHODS: Twenty-three adult patients diagnosed with SSc were randomized 1:1 POM:placebo (PBO). RESULTS: Mean change at Week 52 from baseline in predicted FVC% -5.2 and -2...
November 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/29056770/assessment-of-hospitalization-and-mortality-of-scleroderma-in-patients-a-thirteen-year-study
#18
Saeedeh Shenavandeh, Razieh Naseri
OBJECTIVE: Systemic sclerosis (SSc) is an uncommon non-hereditary sporadic disease that increases the risk of premature death, especially in diffuse type. We determined the prevalence of SSc in the last 13 years in our rheumatologic hospitals as a referral center for southern Iranian patients, the causes of hospitalization, the average length of stay (LOS), the mortality rate, and the reason for their mortality. MATERIAL AND METHODS: A cross-sectional study was performed in Shiraz University of Medical Sciences, Iran...
2017: Reumatologia
https://www.readbyqxmd.com/read/29055411/early-life-gut-dysbiosis-a-driver-of%C3%A2-later-life-fibrosis
#19
Karen J Ho, John Varga
Using a novel mouse model of scleroderma induced by immunization with topoisomerase-I peptide-loaded dendritic cells, Mehta et al. found that early-life antibiotic exposure resulted in increased later-life fibrosis in the skin and lungs. These observations advance the novel concept that gut microbiome alterations caused by early-life exposures may contribute to scleroderma pathogenesis, and warrant in-depth characterization and validation in complementary disease models.
November 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28994016/cardiopulmonary-manifestations-of-collagen-vascular-diseases
#20
REVIEW
Hamza Jawad, Sebastian R McWilliams, Sanjeev Bhalla
PURPOSE OF REVIEW: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. RECENT FINDINGS: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population...
October 9, 2017: Current Rheumatology Reports
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