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Scleroderma

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https://www.readbyqxmd.com/read/28811075/regulatory-t-cell-deficient-scurfy-mice-exhibit-a-th2-m2-like-inflammatory-response-in-the-skin
#1
Stefanie Haeberle, Verena Raker, Jessica Haub, Yong O Kim, Shih-Yen Weng, Osman K Yilmaz, Alexander Enk, Kerstin Steinbrink, Detlef Schuppan, Eva N Hadaschik
BACKGROUND: Scurfy mice have a functional defect in regulatory T cells (Treg), which leads to lethal multi-organ inflammation. The missing Treg function results in uncontrolled autoimmune cellular and humoral inflammatory responses. We and others have previously shown that during the course of disease scurfy mice develop severe skin inflammation and autoantibodies including anti-nuclear autoantibodies (ANA). OBJECTIVE: Autoimmune skin inflammation and ANA are hallmarks for the diagnosis of autoimmune connective tissue diseases; therefore we analyzed scurfy mice for typical signs of these diseases...
July 13, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28805016/microvascular-abnormalities-assessed-by-nailfold-capillaroscopy-in-juvenile-dermatomyositis-after-medium-to-long-term-follow-up
#2
Zoltan Barth, Birgit N Witczak, Berit Flatø, Akos Koller, Ivar Sjaastad, Helga Sanner
OBJECTIVE: In juvenile dermatomyositis (JDM), microvascular abnormalities measured by nailfold capillaroscopy (NFC) are common early in disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to longstanding JDM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. METHODS: Fifty-eight JDM patients clinically examined median 16.8 (2-38) years after disease onset were compared with matched controls...
August 13, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28800024/the-roles-of-dermal-white-adipose-tissue-loss-in-scleroderma-skin-fibrosis
#3
Roberta G Marangoni, Theresa T Lu
PURPOSE OF REVIEW: Dermal white adipose tissue (DWAT) is distinct from subcutaneous white adipose tissue and is lost in scleroderma skin fibrosis. The roles of DWAT loss in scleroderma skin fibrosis have not been well understood, and here we discuss recent findings that begin to provide insight into the multiple mechanisms involved. RECENT FINDINGS: The DWAT loss in part reflects the direct contribution of DWAT cells to the fibrotic tissue, with the reprogramming of adipocytes to myofibroblasts...
August 10, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28799975/diagnosing-and-managing-sclerodermarelated-pulmonary-arterial-hypertension
#4
Katherine Alexis Athanasiou, Sonu Sahni, Amrinder Rana, Arunabh Talwar
Scleroderma is an uncommon autoimmune disease of unknown cause that may affect any organ system in the body. Patients with scleroderma are prone to developing pulmonary complications, including pulmonary arterial hypertension (PAH), that are the leading cause of death in this population. This article describes scleroderma-related PAH and its diagnosis and management.
August 10, 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28795109/edible-wild-mushrooms-of-the-western-ghats-data-on-the-ethnic-knowledge
#5
Namera C Karun, Kandikere R Sridhar
The edible wild mushrooms are most important in food security of ethnic groups and tribals throughout the world. Various indigenous strategies are followed to trace wild mushrooms suitable for human consumption. Data presented in this article projects ethnic knowledge on 51 edible wild mushrooms (in 23 genera) in the Western Ghats region of India. Information collected with support of ethnic groups/tribals pertains to habitats, substrates, mutualistic association, extent of availability, extent of edibility and method of processing of wild mushrooms...
October 2017: Data in Brief
https://www.readbyqxmd.com/read/28780880/-vitamin-d-metabolism-and-osteoporosis-in-systemic-sclerosis
#6
Szilvia Szamosi, Ágnes Horváth, Zoltán Szekanecz, Gabriella Szűcs
In the past few years more and more data have become available on the important role of vitamin D in immunological processes and inflammation. The role of vitamin D deficiency in the pathogenesis as well as in disease progression of different autoimmune and inflammatory conditions is suspected. Vitamin D deficiency is prevalent in several autoimmune diseases, including systemic sclerosis. Hypovitaminosis has been found to be associated with low bone mineral density and higher prevalence of osteoporosis in this group of patients...
August 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28772040/autoimmune-myocarditis-in-systemic-sclerosis-an-unusual-form-of-scleroderma-heart-disease-presentation
#7
Ana Rita Ramalho, Susana Costa, Francisco Silva, Paulo Donato, Fátima Franco, Guilherme Mariano Pêgo
Primary cardiac involvement in systemic sclerosis is common, often subclinical, and is associated with significant mortality. We report the case of a patient who developed autoimmune myocarditis at an early stage of systemic sclerosis, who completely recovered from cardiac dysfunction under optimal medical therapy for heart failure and immunosuppression. This challenging case aims at increasing awareness around the fact that the heart is a target organ of scleroderma disease. It also highlights the importance of screening and early diagnosis of cardiac involvement, because a timely treatment may impact the quality of life of these patients and improve their prognosis...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28761806/scleroderma-related-interstitial-lung-disease
#8
Sally Suliman, Abdalhamid Al Harash, William Neil Roberts, Rafael L Perez, Jesse Roman
Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28752678/risk-factors-and-outcome-of-thai-patients-with-scleroderma-renal-crisis-a-disease-duration-matched-case-control-study
#9
Suparaporn Wangkaew, Supawita Lertthanaphok, Saowanee Puntana, Kajohnsak Noppakun
INTRODUCTION: Data regarding the prevalence, risk factors and outcome of scleroderma renal crisis (SRC) in Asian patients with systemic sclerosis (SSc) are limited. OBJECTIVE: To determine the prevalence, risk factors and outcomes of SRC in Thai SSc patients. METHOD: Medical records of all SSc patients seen at the Division of Rheumatology, Chiang Mai University, Thailand, from January 1990 to December 2015 were retrospectively reviewed. For each SRC case, a disease duration (±1 year) matched control (four SSc patient without SRC for each SRC patient) was identified...
July 27, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28726006/microrna-21-in-skin-fibrosis-potential-for-diagnosis-and-treatment
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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 antifibrotic 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
#18
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
#19
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
#20
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
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