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Scleroderma

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https://www.readbyqxmd.com/read/28544788/fibrosing-myopathy-in-systemic-sclerosis-associates-with-higher-mortality
#1
Julie J Paik, Fredrick M Wigley, Ami A Shah, Andrea M Corse, Livia Casciola-Rosen, Laura K Hummers, Andrew L Mammen
OBJECTIVE: To determine if a unique subtype of scleroderma muscle disease exists by comparing the clinical features of systemic sclerosis (SSc) patients with predominant fibrosis on muscle biopsy to those with inflammatory muscle histopathology. METHODS: This retrospective, cross-sectional study included SSc patients with muscle weakness and an available muscle biopsy. Biopsies with fibrosis but without inflammation/necrosis were designated as "fibrosing myopathy" and those with inflammation and/or necrosis were assigned a category of "inflammatory myopathy"...
May 23, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28544580/efficacy-of-mycophenolate-mofetil-and-oral-cyclophosphamide-on-skin-thickness-post-hoc-analyses-from-the-scleroderma-lung-study-i-and-ii
#2
Rajaie Namas, Donald P Tashkin, Daniel E Furst, Holly Wilhalme, Chi-Hong Tseng, Michael D Roth, Suzanne Kafaja, Elizabeth Volkmann, Philip J Clements, Dinesh Khanna
OBJECTIVES: To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on the modified Rodnan skin score (mRSS) in participants enrolled in the Scleroderma Lung Study (SLS)-I and II. METHODS: SLS-I participants received daily oral CYC or matching placebo for one year, whereas SLS-II participants received daily MMF for 2 years or daily oral CYC for 1 year followed by placebo for second year. We assessed the impact of MMF and CYC on the mRSS in SLS-II over 24-month period...
May 23, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28543515/thrombocytopenia-associated-with-localized-scleroderma-report-of-four-pediatric-cases-and-review-of-the-literature
#3
Ankur Kumar Jindal, Anju Gupta, Sunil Dogra, Amit Rawat, Deepti Suri, Jasmina Ahluwalia, Surjit Singh
We report on four children with localized scleroderma (morphea) and thrombocytopenia. All four had the en coup de sabre subtype of morphea and had varying degrees of thrombocytopenia (8 × 10(9) /L to 120 × 10(9) /L). None of them had major bleeding manifestations, and thrombocytopenia resolved with treatment of morphea. (One patient was also given an injection of anti-D immunoglobulin.) We propose that thrombocytopenia associated with localized scleroderma is usually benign and requires no specific therapy...
May 23, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28543394/bullous-morphoea-a-retrospective-study
#4
M Venturi, A L Pinna, L Pilloni, L Atzori, C Ferreli, F Rongioletti
Bullous morphoea is a rare variant of localized scleroderma whose pathogenesis has been widely discussed. We retrospectively reviewed the records of all histopathologically confirmed cases of morphoea followed from 2005 to 2015 at the Dermatology Clinic and Pathology Institute of the University of Cagliari, Sardinia, Italy. Among 137 patients with morphoea, 2 cases of the bullous variant were identified, which were successfully treated with methotrexate. Thus, the bullous form comprised 1.4% of all cases of morphoea, which is much lower than the 7...
May 22, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28542177/mycophenolate-mofetil-for-scleroderma-related-interstitial-lung-disease-a-real-world-experience
#5
Misbah Baqir, Ashima Makol, Thomas G Osborn, Brian J Bartholmai, Jay H Ryu
BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) remains the number one cause of mortality in scleroderma (SSc). Our goal was to determine the effectiveness of mycophenolate mofetil (MMF) in treating SSc-ILD in a retrospective study. METHODS: A retrospective, computer-assisted search was performed to identify patients with SSc-ILD treated with MMF from 1997 through 2014. We used a novel software tool, Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER), to quantify parenchymal lung abnormalities on high-resolution computed tomography...
2017: PloS One
https://www.readbyqxmd.com/read/28541450/macrocirculation-versus-microcirculation-and-digital-ulcers-in-systemic-sclerosis-patients-macro-microcirculation-and-scleroderma
#6
Maurizio Cutolo, Barbara Ruaro, Vanessa Smith
No abstract text is available yet for this article.
May 23, 2017: Rheumatology
https://www.readbyqxmd.com/read/28540270/fgf-23-klotho-and-vitamin-d-levels-in-scleroderma
#7
Ravan Ahmadi, Mehrzad Hajialilo, Amir Ghorbanihaghjo, Ali Mota, Sina Raeisi, Nasrin Bargahi, Mohammad Valilo, Farahnaz Askarian
BACKGROUND: Scleroderma is a chronic connective tissue disease of unknown etiology. Vitamin D and parathyroid hormone (PTH) that play particular functions in calcium and phosphate homeostasis may be involved in the etiology of this disorder. Klotho, the co-receptor of the fibroblast growth factor 23 (FGF-23), can interfere with calcium and phosphate metabolism. The purpose of this study was to evaluate serum Klotho, FGF-23, intact PTH (iPTH) and vitamin D levels in scleroderma patients compared with the healthy controls...
April 2017: Iranian Journal of Public Health
https://www.readbyqxmd.com/read/28539684/coexistence-of-diabetes-mellitus-type-1-with-diffuse-systemic-sclerosis-case-report-and-literature-review
#8
Ewa Wielosz, Maria Kurowska, Dorota Suszek, Maria Majdan
Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud's phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. The patient presented a typical capillaroscopic scleroderma-like pattern, antinuclear antibodies and sclerotic lesions in gastrointestinal system...
2017: Reumatologia
https://www.readbyqxmd.com/read/28538897/do-you-know-this-syndrome-werner-syndrome
#9
Özlem Bilgiç
Werner syndrome is a rare autosomal recessive disorder, caused by mutations in the WRN gene. Clinical findings include: senile appearance, short stature, grey hair, alopecia, bird-like face, scleroderma-like skin changes, skin ulcers, voice abnormalities, cataracts, osteoporosis, type 2 diabetes mellitus, ischemic heart disease and hypogonadism. The syndrome begins to become apparent in adolescence but it is usually diagnosed in the third or fourth decade of life. Since the patients usually die by the age of 40-50 years related to malignant neoplasms or atherosclerotic complications, they should be closely followed and treated for complications...
March 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28534076/serum-prolidase-activity-in-systemic-sclerosis
#10
Ahmet Celik, Muhammed Nur Birer, Metin Kilinc
Systemic sclerosis, also known as scleroderma, is a complex systemic inflammatory autoimmune disease that targets the vasculature and connective tissue-producing cells and components of the innate and adaptive immune systems. The disease is characterized by a hardening of the skin and an increased synthesis of collagen . Prolidase is a specific imidodipeptidase involved in collagen degradation. The aim of this study was to search the serum prolidase activity (SPA) in the two subtypes of systemic sclerosis: diffuse and limited cutaneous systemic sclerosis...
May 22, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28532772/echocardiographic-predictors-for-worsening-of-six-minute-walk-distances-in-patients-with-systemic-sclerosis-scleroderma
#11
Kenya Kusunose, Hirotsugu Yamada, Susumu Nishio, Yukina Hirata, Hiromitsu Seno, Yoshihito Saijo, Takayuki Ise, Takeshi Tobiume, Koji Yamaguchi, Shusuke Yagi, Takeshi Soeki, Tetsuzo Wakatsuki, Masataka Sata
Change in 6-minute walk distance (6MWD) has been used as a clinical marker in pulmonary hypertension. Determinants and worsening of 6MWD remain a matter of debate because nonpulmonary factors have an impact on the 6MWD. We hypothesized that future reduction of 6MWD in patients with systemic sclerosis (SSc) was more closely associated with cardiac dysfunction. We prospectively performed standard clinical and echocardiographic evaluations in SSc patients with the 6-minute walk test at enrollment. Features associated with the 6MWD were sought in a multiple linear regression analysis and compared using standardized β...
April 26, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28532580/sirt1-ameliorates-systemic-sclerosis-by-targeting-the-mtor-pathway
#12
Xiaoxia Zhu, Haiyan Chu, Shuai Jiang, Qingmei Liu, Lei Liu, Yu Xue, Shucong Zheng, Weiguo Wan, Jianhua Qiu, Jiucun Wang, Hejian Zou
BACKGROUND: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by inflammation and fibrosis. Our previous research has indicated that Sirtuin1 (Sirt1) plays a role in the regulation of TNF-α-induced inflammation; however, whether Sirt1 may inhibit the progress of SSc by blocking inflammation remains unknown. OBJECTIVE: We aimed to investigate the function of Sirt1 in SSc. METHODS: The function and its mechanism of Sirt1 were evaluated in fibroblasts or scleroderma mice...
May 3, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28530671/cardiopulmonary-assessment-of-patients-with-systemic-sclerosis-for-hematopoietic-stem-cell-transplantation-recommendations-from-the-european-society-for-blood-and-marrow-transplantation-autoimmune-diseases-working-party-and-collaborating-partners
#13
REVIEW
D Farge, R K Burt, M-C Oliveira, E Mousseaux, M Rovira, Z Marjanovic, J de Vries-Bouwstra, N Del Papa, R Saccardi, S J Shah, D C Lee, C Denton, T Alexander, D G Kiely, J A Snowden
Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. In SSc, the main cause of non-transplant and TRM is cardiac related. We therefore updated the previously published guidelines for cardiac evaluation, which should be performed in dedicated centers with expertize in HSCT for SSc...
May 22, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28528991/craniofacial-linear-scleroderma-associated-with-retinal-telangiectasia-and-exudative-retinal-detachment
#14
Eva Lenassi, Grace Vassallo, Elias Kehdi, Alice S Chieng, Jane L Ashworth
No abstract text is available yet for this article.
May 18, 2017: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28526340/unfolding-the-pathogenesis-of-scleroderma-through-genomics-and-epigenomics
#15
REVIEW
Pei-Suen Tsou, Amr H Sawalha
With unknown etiology, scleroderma (SSc) is a multifaceted disease characterized by immune activation, vascular complications, and excessive fibrosis in internal organs. Genetic studies, including candidate gene association studies, genome-wide association studies, and whole-exome sequencing have supported the notion that while genetic susceptibility to SSc appears to be modest, SSc patients are genetically predisposed to this disease. The strongest genetic association for SSc lies within the MHC region, with loci in HLA-DRB1, HLA-DQB1, HLA-DPB1, and HLA-DOA1 being the most replicated...
May 16, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28516877/validity-of-the-workers-productivity-and-activity-impairment-questionnaire-specific-health-problem-wpai-shp-in-patients-with-systemic-sclerosis
#16
Kathleen Morrisroe, Wendy Stevens, Molla Huq, Joanne Sahhar, Gene-Siew Ngian, Jane Zochling, Janet Roddy, Susanna M Proudman, Mandana Nikpour
OBJECTIVES: To evaluate the construct validity of the Workers Productivity and Impairment Activity Index: Specific Health Problem (WPAI:SHP) in Australian systemic sclerosis (SSc) patients. METHODS: SSc patients, identified through the Australian Scleroderma Cohort Study database, completed the WPAI:SHP and a quality of life instrument (PROMIS-29) cross-sectionally. The construct validity of the WPAI:SHP was assessed by the correlations between the WPAI:SHP and a range of SSc health states...
May 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28516167/standardization-of-the-modified-rodnan-skin-score-for-use-in-clinical-trials-of-systemic-sclerosis
#17
Dinesh Khanna, Daniel E Furst, Philip J Clements, Yannick Allanore, Murray Baron, Lazlo Czirjak, Oliver Distler, Ivan Foeldvari, Masataka Kuwana, Marco Matucci-Cerinic, Maureen Mayes, Thomas Medsger, Peter A Merkel, Janet E Pope, James R Seibold, Virginia Steen, Wendy Stevens, Christopher P Denton
The modified Rodnan skin score (mRSS) is a measure of skin thickness and is used as a primary or secondary outcome measure in clinical trials of systemic sclerosis (scleroderma). This state-of-art review provides a historical perspective of the development of the mRSS, summarizes the performance of mRSS as an outcome measure, provides guidance on assessing mRSS, and makes recommendations for incorporation of the mRSS into clinical trials.
January 2017: Journal of Scleroderma and related Disorders
https://www.readbyqxmd.com/read/28502960/circulating-anti-nuclear-antibodies-in-systemic-sclerosis-utility-in-diagnosis-and-disease-subsetting
#18
Masataka Kuwana
The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/28499034/functional-disability-and-its-predictors-in-systemic-sclerosis-a-study-from-the-desscipher-project-within-the-eustar-group
#19
Veronika K Jaeger, Oliver Distler, Britta Maurer, Laszlo Czirják, Veronika Lóránd, Gabriele Valentini, Serena Vettori, Francesco Del Galdo, Giuseppina Abignano, Christopher Denton, Svetlana Nihtyanova, Yannick Allanore, Jerome Avouac, Gabriele Riemekasten, Elise Siegert, Dörte Huscher, Marco Matucci-Cerinic, Serena Guiducci, Marc Frerix, Ingo H Tarner, Beata Garay Toth, Beat Fankhauser, Jörg Umbricht, Anastasia Zakharova, Carina Mihai, Franco Cozzi, Sule Yavuz, Nicolas Hunzelmann, Simona Rednic, Alessandra Vacca, Tim Schmeiser, Valeria Riccieri, Paloma García de la Peña Lefebvre, Armando Gabrielli, Brigitte Krummel-Lorenz, Duska Martinovic, Codrina Ancuta, Vanessa Smith, Ulf Müller-Ladner, Ulrich A Walker
Objectives.: The multisystem manifestations of SSc can greatly impact patients' quality of life. The aim of this study was to identify factors associated with disability in SSc. Methods.: SSc patients from the prospective DeSScipher cohort who had completed the scleroderma health assessment questionnaire (SHAQ), a disability score that combines the health assessment questionnaire and five visual analogue scales, were included in this analysis. The effect of factors possibly associated with disability was analysed with multiple linear regressions...
May 11, 2017: Rheumatology
https://www.readbyqxmd.com/read/28496534/collagenous-gastritis-in-a-young-female-with-iga-deficiency
#20
Muhammad S Anwar, Ali Aamar, Ali Marhaba, Jagmohan S Sidhu
Collagenous gastritis, without colonic involvement, is exceptionally rare. It is not known to be associated with IgA deficiency and scleroderma. This is the first report of this type of association. We present a 26-year-old white female with a past medical history of gastroesophageal reflux disease and scleroderma. She was evaluated for complaints of abdominal pain and diarrhea. Esophagogastroduodenoscopy showed gastritis and duodenitis. Colonoscopy was normal. The histopathological report showed collagenous gastritis and focal lymphocytic duodenitis...
April 2017: Gastroenterology Research
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