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Inflammatory myopathy

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https://www.readbyqxmd.com/read/28915335/ecm-related-myopathies-and-muscular-dystrophies-pros-and-cons-of-protein-therapies
#1
Pam M Van Ry, Tatiana M Fontelonga, Pamela Barraza-Flores, Apurva Sarathy, Andreia M Nunes, Dean J Burkin
Extracellular matrix (ECM) myopathies and muscular dystrophies are a group of genetic diseases caused by mutations in genes encoding proteins that provide critical links between muscle cells and the extracellular matrix. These include structural proteins of the ECM, muscle cell receptors, enzymes, and intracellular proteins. Loss of adhesion within the myomatrix results in progressive muscle weakness. For many ECM muscular dystrophies, symptoms can occur any time after birth and often result in reduced life expectancy...
September 12, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28899124/apparent-diffusion-coefficient-adc-does-not-correlate-with-different-serological-parameters-in-myositis-and-myopathy
#2
Hans-Jonas Meyer, Oliver Ziemann, Malte Kornhuber, Alexander Emmer, Ulf Quäschling, Stefan Schob, Alexey Surov
Background Magnetic resonance imaging (MRI) is widely used in several muscle disorders. Diffusion-weighted imaging (DWI) is an imaging modality, which can reflect microstructural tissue composition. The apparent diffusion coefficient (ADC) is used to quantify the random motion of water molecules in tissue. Purpose To investigate ADC values in patients with myositis and non-inflammatory myopathy and to analyze possible associations between ADC and laboratory parameters in these patients. Material and Methods Overall, 17 patients with several myositis entities, eight patients with non-inflammatory myopathies, and nine patients without muscle disorder as a control group were included in the study (mean age = 55...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28895209/focal-hyperkeratosis-overlying-the-palmar-faces-of-interphalangeal-and-metacarpophalangeal-joints-revealing-a-juvenile-dermatomyositis
#3
C Abasq-Thomas, V Devauchelle, S Fraitag, N Jay, G Gourier, A Le Duc Pennec, E Brenaut, L Misery
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin [1]. It is the commonest of the idiopathic inflammatory myopathies of childhood, comprising 85% of cases. It has an annual incidence estimated to range between 1.9 and 4.1 per million children [2, 3]. This article is protected by copyright. All rights reserved.
September 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#4
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28888072/characterization-of-isolated-amyloid-myopathy
#5
Teerin Liewluck, Margherita Milone
BACKGROUND AND PURPOSE: Amyloid myopathy frequently occurs in the setting of systemic amyloidosis and less commonly in isolation (isolated amyloid myopathy). Anoctaminopathy-5 and dysferlinopathy were recently recognized as causes of isolated amyloid myopathy. The present study aimed to characterize the isolated amyloid myopathy and to compare it with amyloid myopathy associated with systemic amyloidosis. METHODS: We searched Muscle Laboratory database to identify patients with pathologically-confirmed amyloid myopathy and were seen in neurology clinics between January 1998 and September 2016...
September 9, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28887614/the-pro-fibrotic-connective-tissue-growth-factor-ctgf-ccn2-correlates-with-the-number-of-necrotic-regenerative-foci-in-dystrophic-muscle
#6
María Gabriela Morales, María José Acuña, Daniel Cabrera, Roel Goldschmeding, Enrique Brandan
Connective tissue growth factor (CTGF/CCN2) has strong inflammatory and profibrotic activities. Its expression is enhanced in skeletal muscular dystrophies such as Duchenne muscular dystrophy (DMD), a myopathy characterized by exacerbated inflammation and fibrosis. In dystrophic tissue, necrotic-regenerative foci, myofibroblasts, newly-regenerated muscle fibers and necrosis all occur simultaneously. To determine if CCN2 is involved in the appearance of the foci, we studied their presence and characteristics in mdx mice (DMD mouse model) compared to mdx mice hemizygous for CCN2 (mdx-Ccn2+/-)...
September 8, 2017: Journal of Cell Communication and Signaling
https://www.readbyqxmd.com/read/28883779/users-knowledge-about-adverse-effects-of-non-steroidal-anti-inflammatory-drugs-in-tirana-albania
#7
Dajana Roshi, Ervin Toçi, Genc Burazeri, Peter Schröder-Bäck, Ledjan Malaj, Helmut Brand
AIM: To assess the level of knowledge of the adult users of non-steroidal anti-inflammatory drugs (NSAIDs) regarding their side effects and the socio-demographic factors associated with the use of these drugs in the adult population of Albania. METHODS: This was a cross-sectional study including a representative sample of 199 individuals aged ≥18 years (77% women; overall mean age: 30.3±10.0 years; overall response rate: 90%) who were users of NSAIDs recruited in ten different pharmacies in Tirana municipality during April-May 2015...
June 2017: Materia Socio-medica
https://www.readbyqxmd.com/read/28879041/pulmonary-arterial-hypertension-a-novelty-in-idiopathic-inflammatory-myopathies-insights-and-first-experiences-with-vasoactive-therapy
#8
Kavish J Bhansing, Anton Vonk-Noordegraaf, Frank Pt Oosterveer, Piet Lcm van Riel, Madelon C Vonk
To characterise the different types of pulmonary hypertension (PH) among idiopathic inflammatory myopathy (IIM). A retrospective case series with assessment of PH by right heart catheterisation, extent of interstitial lung disease (ILD) and outcome of vasoactive therapy.The group of patients with IIM with PH (n=9) showed a median age at PH diagnosis of 62 years (IQR 48-71 years; eight women), seven diagnosed with polymyositis and two with dermatomyositis; median disease duration of 5.7 years and five patients with a positive anti-Jo1 antibody...
2017: RMD Open
https://www.readbyqxmd.com/read/28872084/mortality-trend-of-inpatients-with-connective-tissue-diseases-2005-2014
#9
Liya Li, Xiaoxia Zuo, Hui Luo, Yisha Li, Yunhui You, Liping Duan, Weiru Zhang, Hongjun Zhao, Tong Li, Wangbin Ning, Yanli Xie, Sijia Liu, Xiaoyun Xie, Ying Jiang, Shiyao Wu, Honglin Zhu, Ya'ou Zhou
To analyze the trend relevant factors leading to death and their patterns over a 10-year period in inpatients with connective tissue diseases (CTDs).
 Methods: All clinical data about death in inpatients with CTDs were retrospectively reviewed between 2005 and 2014 at the Department of Rheumatology and Immunology in Xiangya Hospital of Central South University.
 Results: In the 10-year time period, the overall hospital mortality was 15.68‰. The disease itself accounted for 44.71% of the total causes of death, infection accounted for 42...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28866745/mortality-and-prognostic-factors-in-idiopathic-inflammatory-myositis-a-retrospective-analysis-of-a-large-multicenter-cohort-of-spain
#10
Laura Nuño-Nuño, Beatriz Esther Joven, Patricia E Carreira, Valentina Maldonado-Romero, Carmen Larena-Grijalba, Irene Llorente Cubas, Eva Gloria Tomero, María Carmen Barbadillo-Mateos, Paloma García De la Peña Lefebvre, Lucía Ruiz-Gutiérrez, Juan Carlos López-Robledillo, Henry Moruno-Cruz, Ana Pérez, Tatiana Cobo-Ibáñez, Raquel Almodóvar González, Leticia Lojo, María Jesús García De Yébenes, Francisco Javier López-Longo
The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9...
September 2, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28864670/anti-hmgcr-autoantibodies-in-juvenile-idiopathic-inflammatory-myopathies-identify-a-rare-but-clinically-important-subset-of-patients
#11
(no author information available yet)
No abstract text is available yet for this article.
September 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28855175/infections-and-respiratory-tract-disease-as-risk-factors-for-idiopathic-inflammatory-myopathies-a-population-based-case-control-study
#12
John Svensson, Marie Holmqvist, Ingrid E Lundberg, Elizabeth V Arkema
OBJECTIVES: To investigate the association between infection or respiratory tract disease and future risk of developing idiopathic inflammatory myopathy (IIM). METHODS: A case-control study was performed using Swedish nationwide registers. Adults with newly diagnosed IIM were identified (2002-2011) from the National Patient Register (NPR) and the Swedish Rheumatology Register (n=957). Controls were matched by age, sex and place of residence (n=9476). Outpatient visits and hospitalisations preceding IIM diagnosis indicating infection or respiratory disease were identified from NPR...
August 30, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28855174/the-euromyositis-registry-an-international-collaborative-tool-to-facilitate-myositis-research
#13
James B Lilleker, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, Olivier Benveniste, Maria Giovanna Danieli, Katalin Danko, Nguyen Thi Phuong Thuy, Monica Vazquez-Del Mercado, Helena Andersson, Boel De Paepe, Jan L deBleecker, Britta Maurer, Liza J McCann, Nicolo Pipitone, Neil McHugh, Zoe E Betteridge, Paul New, Robert G Cooper, William E Ollier, Janine A Lamb, Niels Steen Krogh, Ingrid E Lundberg, Hector Chinoy
AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. RESULTS: Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%)...
August 30, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28851540/mast-cell-inhibition-as-a-therapeutic-approach-in-fibrodysplasia-ossificans-progressiva-fop
#14
Tracy A Brennan, Carter M Lindborg, Christian R Bergbauer, Haitao Wang, Frederick S Kaplan, Robert J Pignolo
BACKGROUND: Episodic flare-ups of fibrodysplasia ossificans progressiva (FOP) are characterized clinically by severe, often posttraumatic, connective tissue swelling and intramuscular edema, followed histologically by an intense and highly angiogenic fibroproliferative reaction. This early inflammatory and angiogenic fibroproliferative response is accompanied by the presence of abundant mast cells far in excess of other reported myopathies. RESULTS: Using an injury-induced, constitutively-active transgenic mouse model of FOP we show that mast cell inhibition by cromolyn, but not aprepitant, results in a dramatic reduction of heterotopic ossification...
August 26, 2017: Bone
https://www.readbyqxmd.com/read/28841590/cardiovascular-involvement-in-myositis
#15
Louise P Diederichsen
PURPOSE OF REVIEW: The purpose of this review is to provide an update on cardiovascular involvement in idiopathic inflammatory myopathy (IIM). Studies from the past 18 months are identified and reviewed. Finally, the clinical impact of these findings is discussed. RECENT FINDINGS: Epidemiological studies have revealed an increased risk of myocardial infraction and venous thromboembolism (VTE) - including deep venous thrombosis and pulmonary embolism - in adults with polymyositis or dermatomyositis compared to the general population, even after adjustment for potential confounders...
August 24, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28834869/acute-myopathy-following-intra-muscular-injection-of-compound-betamethasone-a-case-report
#16
Li-Yuan Sun, Xiao-Ling Chu
RATIONALE: We report a case of acute steroid myopathy in a patient with eczema receiving one dose of intra-muscular injection of Compound betamethasone. PATIENT CONCERNS: Acute steroid myopathy (ASM) is usually caused by exogenous corticosteroids, and typically, occurs with therapy using intravenous corticosteroids at high doses. DIAGNOSES: The patient was considered as a diagnosis of acute steroid myopathy. INTERVENTIONS: The patient was treated with non-steroid anti-inflammatory drug and other symptomatic therapy...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28832350/which-nonautoimmune-myopathies-are-most-frequently-misdiagnosed-as-myositis
#17
Andrew L Mammen
PURPOSE OF REVIEW: To discuss the spectrum of nonautoimmune myopathies that may be misdiagnosed as autoimmune myopathy. RECENT FINDINGS: Inherited myopathies, such as dysferlinopathy, calpainopathy, and facioscapulohumeral dystrophy may be misdiagnosed as autoimmune myopathy, especially when they have inflammatory muscle biopsies. Inclusion body myositis is frequently misdiagnosed as polymyositis when rimmed vacuoles are absent on muscle biopsy, and a careful neuromuscular evaluation is not performed...
August 19, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28832349/inclusion-body-myositis-advancements-in-diagnosis-pathomechanisms-and-treatment
#18
Karsten Schmidt, Jens Schmidt
PURPOSE OF REVIEW: To review new advances in inclusion body myositis (IBM) and discuss them in light of current knowledge on diagnosis, pathomechanisms, and treatment perspectives. RECENT FINDINGS: IBM is a treatment refractory inflammatory myopathy in middle-aged patients that leads to a slow, relentlessly progressive muscle weakness, and atrophy. Recent data collections suggest that mortality in IBM patients is somewhat elevated compared with the general population...
August 19, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28831580/multiple-values-of-18-f-fdg-pet-ct-in-idiopathic-inflammatory-myopathy
#19
Yuan Li, Yunshan Zhou, Qian Wang
This study aimed to investigate the multiple values of (18)F-FDG PET/CT in detecting malignant tumors, evaluating myopathy, and determining interstitial lung disease in patients with idiopathic inflammatory myopathy (IIM). We retrospectively analyzed the data of 38 patients who were examined by (18)F-FDG PET/CT and eventually diagnosed as IIM. We also collected the data of another 22 cases with negative PET/CT as the control. Pulmonary HRCT images were acquired simultaneously with regular (18)F-FDG PET/CT imaging for each patient...
August 22, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28819164/hypoxia-triggers-ifn-i-production-in-muscle-implications-in-dermatomyositis
#20
Noemí De Luna, Xavier Suárez-Calvet, Cinta Lleixà, Jordi Diaz-Manera, Montse Olivé, Isabel Illa, Eduard Gallardo
Dermatomyositis is an inflammatory myopathy characterized by symmetrical proximal muscle weakness and skin changes. Muscle biopsy hallmarks include perifascicular atrophy, loss of intramuscular capillaries, perivascular and perimysial inflammation and the overexpression of IFN-inducible genes. Among them, the retinoic-acid inducible gene 1 (RIG-I) is specifically overexpressed in perifascicular areas of dermatomyositis muscle. The aim of this work was to study if RIG-I expression may be modulated by hypoxia using an in vitro approach...
August 17, 2017: Scientific Reports
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