keyword
MENU ▼
Read by QxMD icon Read
search

Inflammatory Myopathies

keyword
https://www.readbyqxmd.com/read/28089741/inflammatory-myopathy-in-a-patient-with-aicardi-gouti%C3%A3-res-syndrome
#1
Birutė Tumienė, Norine Voisin, Eglė Preikšaitienė, Donatas Petroška, Jurgita Grikinienė, Rūta Samaitienė, Algirdas Utkus, Alexandre Reymond, Vaidutis Kučinskas
Aicardi-Goutières syndrome (AGS) is an inflammatory disorder belonging to the recently characterized group of type I interferonopathies. The most consistently affected tissues in AGS are the central nervous system and skin, but various organ systems and tissues have been reported to be affected, pointing to the systemic nature of the disease. Here we describe a patient with AGS due to a homozygous p.Arg114His mutation in the TREX1 gene. The histologically proven inflammatory myopathy in our patient expands the range of clinical features of AGS...
January 9, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28088340/the-role-of-magnetic-resonance-imaging-techniques-in-evaluation-and-management-of-the-idiopathic-inflammatory-myopathies
#2
REVIEW
Jessica Day, Sandy Patel, Vidya Limaye
Magnetic resonance imaging (MRI) is an important tool in the evaluation of neuromuscular disorders. MRI accurately demonstrates muscle oedema, atrophy, subcutaneous pathology and fatty infiltration and also highlights the distribution of muscle involvement. This review examines the role of MRI in evaluation of the idiopathic inflammatory myopathies (IIMs), a heterogeneous group of autoimmune conditions characterised by muscle inflammation and a variety of extra-muscular manifestations. MRI has a clear role in aiding diagnosis of these conditions, guiding muscle biopsy, differentiating subtypes of IIM using a pattern-based approach, and monitoring disease activity in a longitudinal fashion...
November 5, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28077146/childhood-arthritis-and-rheumatology-research-alliance-consensus-clinical-treatment-plans-for-juvenile-dermatomyositis-with-skin-predominant-disease
#3
Susan Kim, Philip Kahn, Angela B Robinson, Bianca Lang, Andrew Shulman, Edward J Oberle, Kenneth Schikler, Megan Lea Curran, Lilliana Barillas-Arias, Charles H Spencer, Lisa G Rider, Adam M Huber
BACKGROUND: Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript...
January 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28075491/review-integrated-classification-of-inflammatory-myopathies
#4
Y Allenbach, O Benveniste, H-H Goebel, W Stenzel
Inflammatory myopathies comprise a multitude of diverse diseases, most often occurring in complex clinical settings. In order to ensure accurate diagnosis, multidisciplinary expertise is required. Here, we propose a comprehensive myositis classification that incorporates clinical, morphological and molecular data as well as autoantibody profile. This review focuses on recent advances in myositis research, in particular, the correlation between autoantibodies and morphological or clinical phenotypes that can be used as the basis for an 'integrated' classification system...
January 11, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28063233/establishment-of-a-new-conditionally-immortalized-human-skeletal-muscle-microvascular-endothelial-cell-line
#5
Hironori Sano, Yasuteru Sano, Eri Ishiguchi, Fumitaka Shimizu, Masatoshi Omoto, Toshihiko Maeda, Hideaki Nishihara, Yukio Takeshita, Shiori Takahashi, Mariko Oishi, Takashi Kanda
In skeletal muscle, the capillaries have tight junctions (TJs) that are structurally similar to those in the blood-brain barrier (BBB) and blood-nerve barrier (BNB). Although many findings have been clarified in the territory of BBB and BNB, few have so far examined the TJs of capillaries in the skeletal muscle. In addition, no in vitro human skeletal muscle microvasculature models have been reported thus far. We newly established a new human skeletal muscle microvascular endothelial cell (HSMMEC) line. HSMMECs were isolated from human skeletal muscle and were infected with retroviruses harboring temperature-sensitive SV40 T antigen and telomerase genes...
January 7, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28060361/preoperative-clinical-features-of-reactivated-of-graves-orbitopathy-after-orbital-decompression
#6
Y J Woo, J W Kim, J S Yoon
PurposeTo investigate the incidence and preoperative clinical features of reactivated Graves' orbitopathy (GO) after orbital decompression.MethodsThis study included patients with GO who underwent orbital decompression for disfiguring proptosis and not compressive optic neuropathy and received postoperative follow-up care for more than 12 months. Patients who experienced active inflammatory signs within 6 months of decompression were excluded from analysis. The demographic characteristics, ophthalmic manifestations, and biochemical parameters of the patients were analyzed for association with reactivation of GO by logistic regression analysis...
January 6, 2017: Eye
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#7
Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28056338/hanac-col4a1-mutation-in-mice-leads-to-skeletal-muscle-alterations-due-to-a-primary-vascular-defect
#8
Simon Guiraud, Tiffany Migeon, Arnaud Ferry, Zhiyong Chen, Souhila Ouchelouche, Marie-Christine Verpont, Yoshikazu Sado, Valérie Allamand, Pierre Ronco, Emmanuelle Plaisier
Collagen IV is a major component of basement membranes (BMs). The α1(IV) chain, encoded by the COL4A1 gene, is expressed ubiquitously and associates with the α2(IV) chain to form the α1α1α2(IV) heterotrimer. Several COL4A1 mutations affecting a conformational domain containing integrin-binding sites are responsible for the systemic syndrome of hereditary angiopathy, nephropathy, aneurysms, and cramps (HANAC). To analyze the pathophysiology of HANAC, Col4a1 mutant mice bearing the p.Gly498Val mutation were generated...
January 2, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28054279/successful-resection-of-liver-metastasis-detected-by-exacerbation-of-skin-symptom-in-a-patient-with-dermatomyositis-accompanied-by-rectal-cancer-a-case-report-and-literature-review
#9
Kosuke Ono, Manabu Shimomura, Kazuhiro Toyota, Atsushi Kagimoto, Naofumi Tsukiyama, Masayuki Shishida, Koichi Oishi, Kazuaki Miyamoto, Satoshi Shibata, Masahiro Ikeda, Seiji Sadamoto, Tadateru Takahashi
BACKGROUND: Dermatomyositis (DM) is a rare syndrome that belongs to the group of idiopathic inflammatory myopathies. The association between DM and malignancy is well recognized, and the severity of DM symptoms has been linked to the progression of metastatic disease. CASE PRESENTATION: We report the case of a 42-year-old man that was diagnosed with dermatomyositis (DM) and rectal cancer. Proctectomy was performed, and DM symptoms were resolved postoperatively. One year and 9 months after the surgery, liver metastasis occurred accompanied by the exacerbation of DM symptom...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28053302/polymyositis-without-beneficial-response-to-steroid-therapy-should-miyoshi-myopathy-be-a-differential-diagnosis
#10
Renata Siciliani Scalco, Paulo José Lorenzoni, David S Lynch, William Alves Martins, Heinz Jungbluth, Ros Quinlivan, Jefferson Becker, Henry Houlden
BACKGROUND Miyoshi myopathy (MM) is an autosomal-recessive muscle disorder caused by mutations in the DYSF gene. Clinical features and histopathological changes in dysferlinopathies may mimic inflammatory myopathies and a high degree of clinical suspicion is required to guide the genetic investigation. CASE REPORT We report the case of a 16-year-old male who presented with severe bilateral calf pain and elevated CK levels (15 000 IU/l) who was on prolonged steroid therapy prompted by the clinical suspicion of inflammatory myopathy...
January 5, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28042053/potential-role-for-the-vdr-agonist-elocalcitol-in-metabolic-control-evidences-in-human-skeletal-muscle-cells
#11
Cristina Antinozzi, Clarissa Corinaldesi, Carla Giordano, Annalinda Pisano, Bruna Cerbelli, Silvia Migliaccio, Luigi Di Luigi, Katia Stefanantoni, Gabriella Barbara Vannelli, Salvatore Minisola, Guido Valesini, Valeria Riccieri, Andrea Lenzi, Clara Crescioli
Vitamin D plays a pivotal role to maintain skeletal muscle integrity and health. Vitamin D deficiency characterizes inflammatory myopathy (IM) and diabetes, often overlapping diseases involving skeletal muscle damage. Vitamin D receptor (VDR) agonists likely exert beneficial effects in both IM and metabolic disturbances. We aim to evaluate in vitro the effect of elocalcitol, a non-hypercalcemic VDR agonist, on the biomolecular metabolic machinery of human skeletal muscle cells (Hfsmc), vs. insulin (I). We analyzed GLUT4, Flotillin-1, Caveolin-3 and Caveolin-1 cell expression/localization; mTOR, AKT, ERK and 4E-BP1 phosphorylation; IL-6 myokine release; VDR expression...
December 29, 2016: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28039312/sarcoplasmic-mxa-expression-a-valuable-marker-of-dermatomyositis
#12
Akinori Uruha, Atsuko Nishikawa, Rie S Tsuburaya, Kohei Hamanaka, Masataka Kuwana, Yurika Watanabe, Shigeaki Suzuki, Norihiro Suzuki, Ichizo Nishino
OBJECTIVE: To evaluate the diagnostic value of myxovirus resistance A (MxA) expression in the cytoplasm of myofibers in the diagnosis of dermatomyositis (DM). METHODS: We assessed the sensitivity and specificity of the sarcoplasmic expression of MxA in muscles with DM by immunohistochemistry in consecutive cases of DM (n = 34) and other idiopathic inflammatory myopathies (n = 120: 8 with polymyositis, 16 with anti-tRNA-synthetase antibody-associated myositis, 46 with immune-mediated necrotizing myopathy, and 50 with inclusion body myositis) and compared them with conventional pathologic hallmarks of DM, including perifascicular atrophy (PFA) and membrane attack complex (MAC) deposition on endomysial capillaries...
December 30, 2016: Neurology
https://www.readbyqxmd.com/read/28032847/molecular-markers-of-systemic-autoimmune-disorders-the-expression-of-mhc-located-hsp70-genes-is-significantly-associated-with-autoimmunity-development
#13
Martina Mišunová, Tana Svitálková, Lenka Pleštilová, Olga Kryštufková, Dana Tegzová, Radka Svobodová, Marketa Hušáková, Michal Tomčík, Radim Bečvář, Jakub Závada, Herman Mann, Libor Kolesár, Antonij Slavčev, Jiri Vencovský, Peter Novota
OBJECTIVES: To analyse the expression regulation of two inducible HSP70 genes - HSPA1A and HSPA1B - located within the major histocompatibility complex (MHC) in patients with various systemic autoimmune diseases and to prove the reliability of MHC-located HSP70 genes as molecular markers reflecting the autoimmune process. METHODS: 94 adult patients with idiopathic inflammatory myopathy (IIM, n=31), systemic lupus erythematosus (SLE, n=31) or systemic sclerosis (SSc, n=32) and 37 healthy individuals were analysed...
December 28, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27991411/one-year-in-review-2016-idiopathic-inflammatory-myopathies
#14
REVIEW
Martina Orlandi, Simone Barsotti, Elisa Cioffi, Sara Tenti, Carmela Toscano, Chiara Baldini, Rossella Neri
Idiopathic inflammatory myopathies (IIM) are a group of rare, acquired, clinically heterogeneous autoimmune inflammatory muscle disorders characterised by muscle weakness and multisystem involvement. Recently, new concepts about pathogenesis, diagnosis and treatment of these complex diseases have been provided. The purpose of this manuscript is to summarise the most relevant literature contributions published over the last year.
November 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27986449/quantification-of-muscular-inflammation-by-18-f-fdg-pet-ct-for-initial-evaluation-and-treatment-response-on-inflammatory-myopathy-a-case-report
#15
J R García, A Jaramillo, E Iglesias, E Inarejos, C Jou, E Riera
No abstract text is available yet for this article.
December 13, 2016: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/27974098/use-of-biologic-agents-in-idiopathic-inflammatory-myopathies-in-sweden-a-descriptive-study-of-real-life-treatment
#16
John Svensson, Marie Holmqvist, Anna Tjärnlund, Maryam Dastmalchi, Balsam Hanna, Sara Magnusson Bucher, Ingrid E Lundberg
OBJECTIVES: Biologic treatment has revolutionised treatment in rheumatology in the last decades. Patients with idiopathic inflammatory myopathies (IIM) have so far only been treated with biologics off-label, with little published follow-up on those who are treated and how they are treated. We therefore set out to characterise the Swedish IIM patients who have been treated with biologics. METHODS: By linking Swedish registers we identified 95 patients with IIM who were treated with biologics between 2000 and 2011...
December 9, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27959849/-relapses-in-inflammatory-myopathies
#17
Blas J Larrauri, Diego S Fernández Romero, Maria Cecilia Juri, Alejandro Malbrán
Most studies about treatment of inflammatory myopathies consist of cross-sectional analyses that do not assess long-term efficacy. In the present study we describe the follow-up of seven patients with inflammatory myopathies, 5 polymyositis and 2 dermatomyositis. We describe their clinical features, follow-up, muscle enzyme levels, and treatment responses. We define the latter as treatment cycles, every one of which end when steroid doses need to be increased or a new immunosuppressive drug has to be added because of clinical worsening or sustained increases in muscle enzyme levels...
2016: Medicina
https://www.readbyqxmd.com/read/27936488/ro52-trim21-deficient-expression-and-function-in-different-subsets-of-peripheral-blood-mononuclear-cells-is-associated-with-a-pro-inflammatory-cytokine-response-in-patients-with-idiopathic-inflammatory-myopathies
#18
D Gómez-Martín, A S Galindo-Feria, A Barrera-Vargas, J Merayo-Chalico, G Juárez-Vega, J Torres-Ruiz, J Alcocer-Varela
The presence of anti-Ro52/Trim21 autoantibodies has been associated with a distinctive clinical profile and has gained value as a prognostic marker in idiopathic inflammatory myopathies (IIM). The aim of the present work was to analyze Ro52/Trim21 expression in different subsets of peripheral blood mononuclear cells (PBMCs) of patients with IIM, as well as the ubiquitination profile and its association with pro-inflammatory cytokine production. We included 18 patients with recent onset IIM and 18 age and gender-matched healthy donors...
December 9, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27935079/high-resolution-manometry-in-patients-with-idiopathic-inflammatory-myopathy-elevated-prevalence-of-esophageal-involvement-and-differences-according-to-autoantibody-status-and-clinical-subset
#19
Maria Casal-Dominguez, Iago Pinal-Fernandez, Marianela Mego, Anna Accarino, Lluis Jubany, Fernando Azpiroz, Albert Selva-O'Callaghan
INTRODUCTION: We studied the high-resolution manometry (HRM) findings in patients with dermatomyositis and polymyositis. METHODS: From 2008 to 2015, we performed a cross-sectional study of myositis patients. A survey of esophageal symptoms and a HRM were analyzed and compared among different clinical and serologic groups. RESULTS: Twenty-four (45%) of the 53 patients that were included in the study had manometric involvement that was not correlated with any esophageal symptom (P =0...
December 9, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27919547/granuloma-formation-in-a-patient-with-gne-myopathy-a-case-report
#20
Keiko Nakamura, Tsuyoshi Hamaguchi, Kenji Sakai, Daisuke Noto, Kenjiro Ono, Yukiko Hayashi, Ichizo Nishino, Masahito Yamada
We report a patient with GNE myopathy with a homozygous mutation (c.1505-4G>A) in GNE gene. The patient recognized progressive weakness of extremities at age 60. Neurological examination at age 65 revealed severe weakness and atrophy in the tibialis anterior muscles and distal predominant moderate weakness in the extremities. Muscle biopsy performed at age 65 showed myopathic changes with rimmed vacuoles, and the noteworthy finding was non-caseating epithelioid cell granuloma formation surrounded by numerous inflammatory cells...
November 18, 2016: Neuromuscular Disorders: NMD
keyword
keyword
82624
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"