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Scleroderma

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https://www.readbyqxmd.com/read/28726006/microrna-21-in-skin-fibrosis-potential-for-diagnosis-and-treatment
#1
REVIEW
Yan Li, Juan Zhang, Yuying Lei, Lechun Lyu, Ruiling Zuo, Ting Chen
Skin fibrosis is a common pathological process characterized by fibroblast proliferation and excessive deposition of extracellular matrix. However, the pathogenesis of the disease is still not clear. Previous studies have shown that microRNA-21 may play pivotal roles in the regulation of a variety of skin fibrosis, including keloid, scleroderma, and hypertrophic scar. In this review, we outline the structure, expression, and regulation of microRNA-21 and its role in fibrotic skin diseases. In future, it may be useful as a prognostic or diagnostic marker...
July 19, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28722530/are-there-risk-factors-for-scleroderma-related-calcinosis
#2
Sneha Pai, Vivien Hsu
OBJECTIVES: Pathogenesis and risk factors of scleroderma associated calcinosis is poorly understood and there is no effective treatment. This study was performed to better understand the prevalence and clinical features associated with calcinosis in a cohort of SSc outpatients. METHODS: In this cross-sectional study, we compared clinical characteristics of SSc patients with (SSc-calcinosis) and without calcinosis (SSc-control) seen in the outpatient Rutgers-RWJ Scleroderma Program between 2012 and 2015...
July 19, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28719392/intestinal-microbiome-in-scleroderma-recent-progress
#3
Elizabeth R Volkmann
PURPOSE OF REVIEW: Our evolving understanding of how gut microbiota affects immune function and homeostasis has led many investigators to explore the potentially pathologic role of gut microbiota in autoimmune diseases. This review will discuss the rapidly advancing field of microbiome research in systemic sclerosis (SSc), an incurable autoimmune disease with significant gastrointestinal morbidity and mortality. RECENT FINDINGS: Recent reports have identified common perturbations in gut microbiota across different SSc cohorts...
July 15, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28712039/cutaneous-manifestations-of-scleroderma-and-scleroderma-like-disorders-a-comprehensive-review
#4
REVIEW
Caterina Ferreli, Giulia Gasparini, Aurora Parodi, Emanuele Cozzani, Franco Rongioletti, Laura Atzori
Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations...
July 16, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28711486/assessment-of-english-french-differential-item-functioning-of-the-satisfaction-with-appearance-scale-swap-in-systemic-sclerosis
#5
Lisa R Jewett, Linda Kwakkenbos, Marie Hudson, Murray Baron, Brett D Thombs
The Satisfaction with Appearance Scale (SWAP) has been used to assess body image distress among people with the rare and disfiguring disease systemic sclerosis (SSc); however, it has not been validated across different languages groups. The objective was to examine differential item functioning of the SWAP among 856 Canadian English- or French-speaking SSc patients. Confirmatory factor analysis was used to evaluate the SWAP two-factor structure (Dissatisfaction with Appearance and Social Discomfort). The Multiple-Indicator Multiple-Cause model was utilized to assess differential item functioning...
July 13, 2017: Body Image
https://www.readbyqxmd.com/read/28705939/changes-in-pulmonary-exercise-haemodynamics-in-scleroderma-a-4-year-prospective-study
#6
Gabor Kovacs, Alexander Avian, Nora Wutte, Franz Hafner, Florentine Moazedi-Fürst, Sonja Kielhauser, Elisabeth Aberer, Marianne Brodmann, Winfried Graninger, Vasile Foris, Andrea Olschewski, Horst Olschewski
Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50)...
July 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28705597/-systemic-sclerosis-and-occupational-difficulties-results-of-a-prospective-study
#7
N Peres, S Morell-Dubois, E Hachulla, P Y Hatron, A Duhamel, D Godard, A S Tellart-Mamert, M Lambert, D Launay, S Fantoni-Quinton
Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. A heteroquestionnaire was offered to scleroderma patients the Lille University Hospital. Data were cross-checked with the clinical database of the National Observatory of scleroderma patients. We used 104 questionnaires of which 84% are women. A total of 62...
July 10, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28703365/phototherapy-in-systemic-sclerosis-review
#8
REVIEW
Suteeraporn Chaowattanapanit, Charoen Choonhakarn, Chingching Foocharoen, Narachai Julanon
Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Skin sclerosis can cause disability, leading to decreasing quality of life. Various systemic anti-fibrotic therapies have been used; however, most have unsatisfactory results...
July 13, 2017: Photodermatology, Photoimmunology & Photomedicine
https://www.readbyqxmd.com/read/28697214/facilitation-of-prosthetic-rehabilitation-in-scleroderma-case-history-report
#9
Azam S Mostafavi, Mohammadreza Hajimahmoudi
Dental management of scleroderma patients is difficult and complicated because of compromised intraoral access. Physiotherapy may play an adjunctive and effective treatment role as described in a follow-up report on a previously documented case history.
July 2017: International Journal of Prosthodontics
https://www.readbyqxmd.com/read/28691464/parry-romberg-syndrome-and-linear-facial-scleroderma-management-in-pediatric-population
#10
E Segna, V Pucciarelli, G A Beltramini, C Sforza, F J Silvestre, A B Giannì, A Baj
Among all different types of cutaneous scleroderma, Parry Romberg syndrome and linear scleroderma “en coup de sabre” typically involve the visage. Gradual degeneration of the tissues, from the skin up to the bone, is the stigmata of the diseases and the range of clinical manifestations is wide. They typically start during childhood and slowly progress before stabilizing. Considering the gravity of the associated deformity and its impact on facial function and appearance, we strongly advocate a prompt intervention that however must be tailored on paediatric patients...
April 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28690947/autoamputation-and-polyneuropathy-in-mixed-connective-tissue-disorder-a-case-report
#11
Syeda Naqvi, Vikash Talib, Razia Aijaz, Zeeshan Ali, Shehroz Bashir, Syed Masroor Ahmad, Shabnam Naveed
Mixed connective tissue disorder (MCTD) is a multisystem disease with overlapping features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), myositis, rheumatoid arthritis, and scleroderma. MCTD presents with a distinctive antibody in serum known as U1-ribonucleoprotein (RNP). MCTD is quite rare as compared to other connective tissue disorders like SLE, systemic sclerosis, dermatomyositis, and polymyositis. We describe a case of MCTD in a young Asian female of 30 year old. This case highlights rare co-existence of polyneuropathy and autoamputation in MCTD disorder...
June 5, 2017: Curēus
https://www.readbyqxmd.com/read/28688535/caballeronia-mineralivorans-sp-nov-isolated-from-oak-scleroderma-citrinum-mycorrhizosphere
#12
S Uroz, P Oger
Six bacterial strains were isolated from the oak-Scleroderma citrinum ectomycorrhizosphere in acidic and nutrient-poor forest soil for their high efficacy to weather minerals. Four of the six isolates, PML1(12)(T) and PML1(4), PML1(14) and PML1(16), were further characterized extensively. They were Gram negative, obligate aerobic, motile, non spore forming and rod-shaped. The major fatty acids of strain PML1(12)(T) were cyclo-C17:0, cyclo-C19:0-ω8c, C16:0 and C18:1-ω7c. The GC content of the DNA was 60.8%...
June 7, 2017: Systematic and Applied Microbiology
https://www.readbyqxmd.com/read/28676069/limited-cutaneous-systemic-sclerosis-skin-demonstrates-distinct-molecular-subsets-separated-by-a-cardiovascular-development-gene-expression-signature
#13
Emma C Derrett-Smith, Viktor Martyanov, Cecilia B Chighizola, Pia Moinzadeh, Corrado Campochiaro, Korsa Khan, Tammara A Wood, Pier Luigi Meroni, David J Abraham, Voon H Ong, Robert Lafyatis, Michael L Whitfield, Christopher P Denton
BACKGROUND: Systemic sclerosis (SSc; scleroderma) is an uncommon autoimmune rheumatic disease characterised by autoimmunity, vasculopathy and fibrosis. Gene expression profiling distinguishes scleroderma from normal skin, and can detect different subsets of disease, with potential to identify prognostic biomarkers of organ involvement or response to therapy. We have performed gene expression profiling in skin samples from patients with limited cutaneous SSc (lcSSc). METHODS: Total RNA was extracted from clinically uninvolved skin biopsies of 15 patients with lcSSc and 8 healthy controls (HC)...
July 4, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28668815/adie-pupil-as-the-initial-presentation-of-localized-en-coup-de-sabre-scleroderma
#14
Ronald M Laxer, Steven Miller, Elena Pope
No abstract text is available yet for this article.
July 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28668810/an-mif-promoter-polymorphism-is-associated-with-susceptibility-to-pulmonary-arterial-hypertension-in-diffuse-cutaneous-systemic-sclerosis
#15
Lara Bossini-Castillo, Diana Campillo-Davó, Elena López-Isac, Francisco David Carmona, Carmen P Simeon, Patricia Carreira, José Luis Callejas-Rubio, Iván Castellví, Antonio Fernández-Nebro, Luis Rodríguez-Rodríguez, Manel Rubio-Rivas, Francisco J García-Hernández, Ana Belén Madroñero, Lorenzo Beretta, Alessandro Santaniello, Claudio Lunardi, Paolo Airó, Anna-Maria Hoffmann-Vold, Alexander Kreuter, Gabriela Riemekasten, Torsten Witte, Nicolas Hunzelmann, Madelon C Vonk, Alexandre E Voskuyl, J de Vries-Bouwstra, Paul Shiels, Ariane Herrick, Jane Worthington, Timothy R D J Radstake, Javier Martin
OBJECTIVE: Systemic sclerosis (SSc) is a fibrotic immune-mediated disease of unknown etiology. Among its clinical manifestations, pulmonary involvement is the leading cause of mortality in patients with SSc. However, the genetic factors involved in lung complication are not well defined. We aimed to review the association of the MIF gene, which encodes a cytokine implicated in idiopathic pulmonary hypertension among other diseases, with the susceptibility and clinical expression of SSc, in addition to testing the association of this polymorphism with SSc-related pulmonary involvement...
July 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28667931/the-impact-of-high-dose-narrowband-ultraviolet-a1-on-dermal-thickness-collagen-and-matrix-metalloproteinases-in-animal-model-of-scleroderma
#16
Diana Karpec, Romualdas Rudys, Laima Leonaviciene, Zygmunt Mackiewicz, Ruta Bradunaite, Gailute Kirdaite, Algirdas Venalis
OBJECTIVE: The main purpose of the present study was to define the impact of high-dose of 365±5nm ultraviolet A1 (UVA1) on dermal fibrosis in the pre-established, bleomycin-induced mouse model of scleroderma. METHODS: DBA/2 strain mice with the pre-established, bleomycin-induced scleroderma were irradiated with cumulative UVA1 dose of 1200J/cm(2) and in parallel were challenged with prolonged administration of bleomycin. Non-treated groups served as the control...
June 21, 2017: Journal of Photochemistry and Photobiology. B, Biology
https://www.readbyqxmd.com/read/28664833/significance-of-pentraxin-3-in-patients-with-juvenile-scleroderma
#17
Amra Adrovic, Sezgin Sahin, Kenan Barut, Sinem Durmus, Hafize Uzun, Ozgur Kasapcopur
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June 20, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28664832/clinical-determinants-of-elevated-systolic-pulmonary-artery-pressure-measured-by-transthoracic-doppler-echocardiography-in-early-systemic-sclerosis
#18
Patricia E Carreira, Loreto Carmona, Beatriz E Joven, Estibaliz Loza, Jose Luis Andreu, Gabriela Riemekasten, Serena Vettori, Yannick Allanore, Alexandra Balbir-Gurman, Paolo Airò, Ulrich A Walker, Nemanja Damjanov, Lidia P Ananieva, Simona Rednic, László Czirják, Oliver Distler, Dominique Farge, Roger Hesselstrand, Ada Corrado, Paola Caramaschi, Mohammed Tikly, Marco Matucci-Cerinic
OBJECTIVES: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc). METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected...
June 20, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28661697/improvement-in-right-ventricular-strain-with-ambrisentan-and-tadalafil-upfront-therapy-in-scleroderma-pulmonary-arterial-hypertension
#19
Valentina Mercurio, Monica Mukherjee, Ryan J Tedford, Roham T Zamanian, Rubina M Khair, Takahiro Sato, Omar A Minai, Fernando Torres, Reda E Girgis, Kelly Chin, Rachel Damico, Todd M Kolb, Stephen C Mathai, Paul M Hassoun
No abstract text is available yet for this article.
June 29, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28654764/deep-proteome-profiling-reveals-common-prevalence-of-mzb1-positive-plasma-b-cells-in-human-lung-and-skin-fibrosis
#20
Herbert B Schiller, Christoph H Mayr, Gabriela Leuschner, Maximilian Strunz, Claudia Staab-Weijnitz, Stefan Preisendörfer, Beate Eckes, Pia Moinzadeh, Thomas Krieg, David A Schwartz, Rudolf A Hatz, Jürgen Behr, Matthias Mann, Oliver Eickelberg
RATIONALE: Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets. OBJECTIVES: We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across endstage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis...
June 27, 2017: American Journal of Respiratory and Critical Care Medicine
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