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Dermatomyositis

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https://www.readbyqxmd.com/read/29774953/dermoscopic-patterns-of-filiform-papillae-of-the-tongue-in-patients-with-and-without-connective-tissue-autoimmune-diseases
#1
José Manuel Díaz-González, María E Vega-Memije, Adalberto Mosqueda-Taylor, Víctor Noé García-Edgar, Juan Carlos Cuevas-González
BACKGROUND: Connective tissue autoimmune diseases (CTADs) constitute a group of conditions, including rheumatoid arthritis; systemic lupus erythematosus; mixed connective tissue disease; calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome; scleroderma; dermatomyositis; and Sjögren syndrome. There are few studies on the alterations in filiform papillae in CTAD. Thus, the objective of this work was to determine whether there are changes in the macroscopic and dermoscopic patterns of filiform papillae...
May 18, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29773272/juvenile-dermatomyositis-latest-advances
#2
REVIEW
Qiong Wu, Lucy R Wedderburn, Liza J McCann
Registries and biobanks for juvenile dermatomyositis (JDM) have generated statistical power to help understand pathogenesis and determine treatment and long-term outcomes in this rare and heterogeneous disease. Genotype, autoantibodies, muscle histology and early clinical features may predict prognosis and guide personalised treatment. While corticosteroids and disease-modifying anti-rheumatic drugs improve outcomes, there remain children who experience refractory disease. Ongoing research into the aberrant immune response and novel biological targets is necessary...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773266/genetic-interferonopathies-an-overview
#3
REVIEW
Despina Eleftheriou, Paul A Brogan
Interferonopathies comprise an expanding group of monogenic diseases characterised by disturbance of the homeostatic control of interferon (IFN)-mediated immune responses. Although differing in the degree of phenotypic expression and severity, the clinical presentation of these diseases shows a considerable degree of overlap, reflecting their common pathogenetic mechanisms. Increased understanding of the molecular basis of these Mendelian disorders has led to the identification of targeted therapies for these diseases, which could also be of potential relevance for non-genetic IFN-mediated diseases such as systemic lupus erythematosus and juvenile dermatomyositis...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773230/medications-received-by-patients-with-juvenile-dermatomyositis
#4
Takayuki Kishi, Nastaran Bayat, Michael M Ward, Adam M Huber, Lan Wu, Gulnara Mamyrova, Ira N Targoff, William J Warren-Hicks, Frederick W Miller, Lisa G Rider
OBJECTIVE: Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM. METHODS: We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the association of demographic and clinical features and autoantibodies with medication usage...
March 28, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29772037/a-review-of-fabry-disease
#5
A Cinats, E Heck
The class of medications known as Janus kinase inhibitors block cytokine-mediated signaling via the Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway, which plays an important role in immunoregulation and normal cell growth. This class includes the drugs tofacitinib, approved for the treatment of rheumatoid arthritis, and ruxolitinib, approved for the treatment of myelofibrosis and polycythemia rubra vera. The most common adverse events (AEs) reported in patients taking tofacitinib are infections, whereas the most common AEs in patients taking ruxolitinib are anemia and thrombocytopenia...
May 2018: Skin Therapy Letter
https://www.readbyqxmd.com/read/29770465/expression-of-myxovirus-resistance-protein-a-a-possible-marker-of-muscle-disease-activity-and-autoantibody-specificities-in-juvenile-dermatomyositis
#6
Sirisucha Soponkanaporn, Claire T Deakin, Peter W Schutz, Lucy R Marshall, Shireena A Yasin, Cerise M Johnson, Erdal Sag, Sarah L Tansley, Neil J McHugh, Lucy R Wedderburn, Thomas S Jacques
AIMS: To evaluate the relationship between expression of Myxovirus-resistance protein A (MxA) protein on muscle biopsies by immunohistochemistry and disease activity in JDM patients. Also, another aim was to investigate whether the expression of MxA is related with myositis-specific autoantibodies (MSA) status in JDM patients. METHODS: 103 patients (median aged 6.3, IQR 0.5-15.9) enrolled in the Juvenile Dermatomyositis Cohort and Biomarker Study (JDCBS). Muscle biopsies were stained with MxA and scored...
May 16, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29766941/idiopathic-inflammatory-myopathies-in-adults-a-comparative-study-of-bohan-and-peter-and-european-neuromuscular-center-2004-criteria
#7
Sundaram Challa, Saumya Jakati, Megha S Uppin, Meena A Kannan, Rajasekhar Liza, M K Murthy Jagarlapudi
Background: Bohan and Peter criteria are widely used for the diagnosis of idiopathic inflammatory myopathies (IIMs). Recently, European Neuromuscular Center (ENMC) formulated criteria to identify subgroups of IIMs. Aim: To compare the two diagnostic criteria in adult IIMs. Materials and Methods: This was a retrospective review of case records of histologically confirmed IIMs in adults between January 2014 and May 2015. Both the Bohan and Peter, and ENMC 2004 criteria were applied in the same group of patients to subgroup the IIMs...
May 2018: Neurology India
https://www.readbyqxmd.com/read/29759882/dermatomyositis
#8
José Ramón Vizán-Caravaca, Ana Isabel Cascales-Vallejo, Marina Aroza-Espinar
No abstract text is available yet for this article.
May 11, 2018: Medicina Clínica
https://www.readbyqxmd.com/read/29750110/non-specific-interstitial-pneumonia-associated-with-clinically-amyopathic-dermatomyositis-showing-crazy-paving-appearance-on-thin-section-lung-ct
#9
Yuya Aono, Tatsuru Eifuku, Tomohiro Uto, Jun Sato, Shiro Imokawa, Takafumi Suda
The "crazy paving" appearance consists of ground-glass opacity superimposed on a network of linear opacities on thin-section computed tomography (CT) images of the lung. This finding has been described in a variety of diseases but is extremely rare in patients with non-specific interstitial pneumonia (NSIP). We describe a 45-year-old woman with biopsy-proven NSIP associated with clinically amyopathic dermatomyositis that showed a "crazy paving" appearance on thin-section CT of the lung. Clinicians should include NSIP in the differential diagnosis in patients presenting with "crazy paving" appearance on thin-section chest CT...
July 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29745890/pattern-of-muscle-involvement-in-inclusion-body-myositis-a-sonographic-study
#10
Jemima Albayda, Lisa Christopher-Stine, Clifton O Bingham Iii, Julie J Paik, Eleni Tiniakou, Seth Billings, O Manny Uy, Philippe Burlina
OBJECTIVES: Imaging plays a role in myositis assessment by detecting muscle changes indicative of pathology. This study was conducted to determine the ultrasonographic pattern of muscle involvement in patients with inclusion body myositis (IBM) through an assessment of muscle echointensity. METHODS: Sixty-two individuals were consecutively studied, 18 with IBM, 16 with polymyositis or dermatomyositis and 28 normal controls. Standardised scans were completed bilaterally for the deltoids, biceps, flexor digitorum profundus (FDP), flexor carpi ulnaris, rectus femoris, tibialis anterior and gastrocnemius assessing for muscle echointensity changes...
May 8, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29745874/strong-correlation-between-cancer-progression-and-anti-transcription-intermediary-factor-1%C3%AE-antibodies-in-dermatomyositis-patients
#11
Mariko Ogawa-Momohara, Yoshinao Muro, Teruyuki Mitsuma, Masao Katayama, Koichi Yanaba, Mizuho Nara, Masao Kakeda, Michihiro Kono, Masashi Akiyama
OBJECTIVES: Transcription intermediary factor 1γ (ΤΙF1γ) protein is known as a tumour suppressor that promotes cellular differentiation. Autoantibodies to ΤΙF1γ have a strong clinical association with cancers associated with dermatomyositis (DM). This study aims to identify the clinical characteristics of cancers in anti-ΤΙF1γ antibody-positive adult patients with DM. METHODS: This retrospective analysis covered 160 adult DM patients who visited Nagoya University Hospital or collaborating medical centres between 2003 and 2016...
May 8, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29741608/jak-inhibitor-improves-type-i-interferon-induced-damage-proof-of-concept-in-dermatomyositis
#12
Leandro Ladislau, Xavier Suárez-Calvet, Ségolène Toquet, Océane Landon-Cardinal, Damien Amelin, Marine Depp, Mathieu P Rodero, Denisa Hathazi, Darragh Duffy, Vincent Bondet, Corinna Preusse, Boris Bienvenu, Flore Rozenberg, Andreas Roos, Claudia F Benjamim, Eduard Gallardo, Isabel Illa, Vincent Mouly, Werner Stenzel, Gillian Butler-Browne, Olivier Benveniste, Yves Allenbach
Dermatomyositis is an acquired auto-immune disease characterized by skin lesions and muscle-specific pathological features such as perifascicular muscle fibre atrophy and vasculopathy. Dermatomyositis patients display an upregulation of type I interferon-inducible genes in muscle fibres, endothelial cells, skin and peripheral blood. However, the effect of type I interferon on muscle tissue has not yet been determined. Our aim was to study the pathogenicity of type I interferon in vitro and to evaluate the efficacy of the type I interferon pathway blockade for therapeutic purposes...
May 8, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29741130/quantitative-characterization-of-the-relationship-between-levels-of-extended-corticosteroid-use-and-related-adverse-events-in-a-us-population
#13
J Bradford Rice, Alan G White, Michaela Johnson, Aneesha Wagh, Yimin Qin, Laura Bartels-Peculis, Gosia Ciepielewska, Winnie W Nelson
OBJECTIVE: This retrospective study assessed the incidence and timing of adverse events (AEs) among patients prescribed varying dose levels of corticosteroids in the US. METHODS: Patients with selected autoimmune or inflammatory disease diagnoses between 2006 and 2015 were identified from a privately-insured administrative database. Patients were stratified into treatment cohorts based on dosage and length of corticosteroid use: intermittent use with duration <60 days, and three extended use cohorts with duration ≥60 days at low (≤7...
May 9, 2018: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/29737059/-the-clinical-significance-of-tumor-associated-antigens-in-dermatomyositis-patients-with-interstitial-lung-disease
#14
Yuan Yang, Jing-Cheng Hao, Yuan Chen, Yi Liu, Qi-Bing Xie, Geng Yin
OBJECTIVE: To investigate the relationship between tumor related markers and the presence of interstitial lung disease (ILD) in dermatomyositis (DM) patients as well as potential serum markers for accompanied ILD. METHODS: Fifty-nine DM patients were included,including 30 patents with ILD. Serum level of anti-transcription intermediary factor1-γ (TIF1-γ) and tumor-associated antigens (TAAs) were detected to analyze the correlation of these markers with ILD. Meanwhile,the diagnostic value of these markers was evaluated by receive operating characteristic (ROC) curve analysis...
March 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/29737058/-risk-factors-of-respiratory-failure-in-the-dermatomyositis-patients-with-interstitial-lung-disease
#15
Su-Han Zhang, Yun Peng, Qi-Bing Xie, Geng Yin, Bing Yan
OBJECTIVE: To reveal the clinical features of respiratory failure (RF) in dermatomyositis (DM) patients with interstitial lung disease (ILD),and to explore risk factors of RF in these patients. METHODS: The medical data of 122 DM patients with ILD were retrospectively reviewed: 40 developed RF (RF group),82 did not develope RF (Non RF group). Clinical,laboratory and radiological variables were compared between RF patients and Non RF patients. Multivariate Logistic regression was used to analyze risk factors of RF...
March 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/29728522/muscular-and-extramuscular-clinical-features-of-patients-with-anti-pm-scl-autoantibodies
#16
Rebecca De Lorenzo, Iago Pinal-Fernandez, Wilson Huang, Jemima Albayda, Eleni Tiniakou, Cheilonda Johnson, Jose C Milisenda, Maria Casal-Dominguez, Andrea M Corse, Sonye K Danoff, Lisa Christopher-Stine, Julie J Paik, Andrew L Mammen
OBJECTIVE: To define the clinical features of myositis patients with anti-PM/Scl-75 and/or anti-PM/Scl-100 autoantibodies at disease onset and during the course of disease and compare them to patients with other forms of myositis. METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up were compared between anti-PM/Scl-positive patients and those with the antisynthetase syndrome (AS), dermatomyositis (DM), and immune-mediated necrotizing myopathy (IMNM)...
May 4, 2018: Neurology
https://www.readbyqxmd.com/read/29721695/inverse-gottron-papules-in-juvenile-dermatomyositis-an-under-recognized-clinical-entity
#17
Ankur Kumar Jindal, Sandesh Guleria, Rakesh Kumar Pilania, Anuradha Bishnoi, Keshavamurthy Vinay, Sunil Dogra, Deepti Suri, Amit Rawat, Surjit Singh
The objective of this study was to report four children having juvenile dermatomyositis (JDM) with inverse Gottron sign along with a brief review of literature of similarly published cases. This is a retrospective review of case records of all children diagnosed with JDM at a single tertiary care centre in North India. Children who were found to have Gottron papules over palmar surface were identified. A total of 127 children were diagnosed with JDM in the last 24 years (1994-2017). Out of these 127 patients, 4 were found to have Gottron papules over palmar surface...
May 2, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29720802/a-viral-polymyositis-masquerade-life-threatening-case-of-juvenile-dermatomyositis-complicated-by-systemic-capillary-leak-syndrome
#18
Bhaskara P Shelley, Shrijeet Chakraborti
This is a case report of an 8-year-old boy who developed an atypical, rare subphenotype of autoimmune inflammatory acute juvenile dermatomyositis (JDM), initially masquerading as viral polymyositis (PM)-like presentation, that was complicated by a hitherto unreported fulminant, life-threatening pediatric systemic capillary leak syndrome (SCLS). We highlight the close differential between viral PM and JDM, the baffling clinical syndromic constellation of hypotension with hemoconcentration - a "shock"-like syndrome, hypoalbuminemia without albuminuria, and generalized edema with the atypical JDM presentation, and stress crucial need to implement early aggressive, multipronged immunomodulatory treatment along with intensive fluid resuscitation which saved the life, this patient from a stormy, and turbulent 4-week clinical illness...
January 2018: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29720222/targeted-lipidomics-analysis-identified-altered-serum-lipid-profiles-in-patients-with-polymyositis-and-dermatomyositis
#19
Joan Raouf, Helena Idborg, Petter Englund, Helene Alexanderson, Maryam Dastmalchi, Per-Johan Jakobsson, Ingrid E Lundberg, Marina Korotkova
BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are severe chronic autoimmune diseases, characterized by muscle fatigue and low muscle endurance. Conventional treatment includes high doses of glucocorticoids and immunosuppressive drugs; however, few patients recover full muscle function. One explanation of the persistent muscle weakness could be altered lipid metabolism in PM/DM muscle tissue as we previously reported. Using a targeted lipidomic approach we aimed to characterize serum lipid profiles in patients with PM/DM compared to healthy individuals (HI) in a cross-sectional study...
May 2, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29713969/rituximab-for-refractory-rapidly-progressive-interstitial-lung-disease-related-to-anti-mda5-antibody-positive-amyopathic-dermatomyositis
#20
Ho So, Victor Tak Lung Wong, Virginia Weng Nga Lao, Hin Ting Pang, Ronald Man Lung Yip
To report our experience in using rituximab (RTX) for treating refractory rapidly progressive interstitial lung disease (RP-ILD) complicating anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive amyopathic dermatomyositis (ADM). Medical records of four ADM patients with refractory RP-ILD treated with RTX therapy were reviewed retrospectively. All four patients were tested positive for anti-MDA5 Ab and failed to respond to high-dose systemic steroid and other intensive immunosuppressive therapies...
April 30, 2018: Clinical Rheumatology
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