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https://www.readbyqxmd.com/read/28096992/development-of-myasthenia-gravis-8-years-after-interstitial-lung-disease-associated-with-antisynthetase-anti-ej-antibody-syndrome
#1
Takashi Ishiguro, Naho Kagiyama, Eriko Kawate, Kyuto Odashima, Yotaro Takaku, Kazuyoshi Kurashima, Noboru Takayanagi
Patients with antisynthetase-positive interstitial lung disease (ILD) alone sometimes develop myositis during follow-up, but myasthenia gravis (MG) overlapping on antisynthetase syndrome is unusual. A 56-year-old woman with ILD and anti-EJ antibody treated for 8 years developed MG. Physicians should consider myositis and MG when patients develop muscle symptoms during follow-up.
January 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28096458/more-severe-disease-and-slower-recovery-in-younger-patients-with-anti-3-hydroxy-3-methylglutaryl-coenzyme-a-reductase-associated-autoimmune-myopathy
#2
Eleni Tiniakou, Iago Pinal-Fernandez, Thomas E Lloyd, Jemima Albayda, Julie Paik, Jessie L Werner, Cassie A Parks, Livia Casciola-Rosen, Lisa Christopher-Stine, Andrew L Mammen
OBJECTIVE: To study disease severity and response to therapy in a large cohort of patients with anti-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR)-associated myositis. METHODS: Muscle strength, creatine kinase levels and treatments were assessed in anti-HMGCR-positive patients at each clinical visit. Univariate and multivariate analyses were used to analyse the influence of clinical characteristics on strength and the change in strength over time. Whole exome sequencing was performed in a subset of patients...
January 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/28095296/diverse-role-of-survival-motor-neuron-protein
#3
REVIEW
Ravindra N Singh, Matthew D Howell, Eric W Ottesen, Natalia N Singh
The multifunctional Survival Motor Neuron (SMN) protein is required for the survival of all organisms of the animal kingdom. SMN impacts various aspects of RNA metabolism through the formation and/or interaction with ribonucleoprotein (RNP) complexes. SMN regulates biogenesis of small nuclear RNPs, small nucleolar RNPs, small Cajal body-associated RNPs, signal recognition particles and telomerase. SMN also plays an important role in DNA repair, transcription, pre-mRNA splicing, histone mRNA processing, translation, selenoprotein synthesis, macromolecular trafficking, stress granule formation, cell signaling and cytoskeleton maintenance...
January 14, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28089977/antimelanoma-differentiation-associated-gene-5-antibody-expanding-the-clinical-spectrum-in-north-american-patients-with-dermatomyositis
#4
Siamak Moghadam-Kia, Chester V Oddis, Shinji Sato, Masataka Kuwana, Rohit Aggarwal
OBJECTIVE: To determine the clinical features associated with the antimelanoma differentiation-associated gene 5 antibody (anti-MDA5) in US patients with clinically amyopathic dermatomyositis (CADM) and classic DM. METHODS: Patients with CADM were consecutively selected from the University of Pittsburgh Myositis Database from 1985 to 2013. CADM was defined by a typical DM rash without objective muscle weakness and no or minimal abnormalities of muscle enzymes, electromyography, or muscle biopsy...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28076913/use-of-intravenous-immunoglobulin-therapy-for-myositis-an-audit-in-south-australian-patients
#5
Caroline Foreman, Paul Russo, Noelene Davies, Pravin Hissaria, Susanna Proudman, Tiffany Hughes, Vidya Limaye
In South Australia, between 2000 and 2014, 57 patients with idiopathic inflammatory myositis (IIM) were treated with intravenous immunoglobulin (IVIg). We reviewed disease characteristics to determine predictors of response to therapy and IVIg dosing and duration to identify opportunities to rationalise IVIg use. Patients with dermatomyositis/polymyositis had a response rate of 77% and were more likely than inclusion body myositis to respond to therapy. Consideration should be given to the use of the lowest possible dose of IVIg and to the undertaking of trials of cessation of IVIg in patients with stable IIM...
January 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28076378/p62-pathology-model-in-the-rat-substantia-nigra-with-filamentous-inclusions-and-progressive-neurodegeneration
#6
Kasey L Jackson, Wen-Lang Lin, Sumitra Miriyala, Robert D Dayton, Manikandan Panchatcharam, Kevin J McCarthy, Monica Castanedes-Casey, Dennis W Dickson, Ronald L Klein
One of the proteins most frequently found in neuropathological lesions is the ubiquitin binding protein p62 (sequestosome 1). Post-mortem analysis of p62 is a defining diagnostic marker in several neurodegenerative diseases including amyotrophic lateral sclerosis and inclusion body myositis. Since p62 functions in protein degradation pathways including autophagy, the build-up of p62-positive inclusions suggests defects in protein clearance. p62 was expressed unilaterally in the rat substantia nigra with an adeno-associated virus vector (AAV9) in order to study p62 neuropathology...
2017: PloS One
https://www.readbyqxmd.com/read/28075491/review-integrated-classification-of-inflammatory-myopathies
#7
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/28074445/intrathoracic-tumor-of-the-chest-wall-a-case-of-castleman-s-disease-mimicking-myositis-of-the-lower-extremities
#8
Athanasios Tampakis, Ekaterini Christina Tampaki, Thomas Daikeler, Didier Lardinois
Castleman's disease refers to a group of uncommon lymphoproliferative disorders which exhibit common lymph-node histological features. A 72-year-old male patient presented with signs of lower limb myositis. Detailed work-up focused initially on evaluating hematological malignancies, the presence of a solid tumor, autoimmune diseases and degenerative disorders of the peripheral nerves. Finally, a PET-CT scan was performed to exclude paraneoplastic manifestations of a primary tumor, revealing  however a tumor of the thoracic wall...
January 10, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28073522/antisynthetase-syndrome-analysis-of-11-cases
#9
Ester Zamarrón-de Lucas, Luis Gómez Carrera, Gema Bonilla, Dessiree Petit, Alberto Mangas, Rodolfo Álvarez-Sala
INTRODUCTION: Antisynthetase syndrome (ASS) is characterised by a series of clinical manifestations such as myositis, fever, mechanic's hands and diffuse interstitial lung disease (ILD), all associated with positivity to antisynthetase antibodies. The presence of ILD will be that, to a great extent it will mark the response to treatment and prognosis. PATIENTS AND METHODS: Eleven cases of patients with ASS and pulmonary involvement in monitoring at a Pulmonary monographic consult in a third level hospital consult are described...
January 7, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28068299/masseteric-myositis-a-unique-cause-of-obstructive-sialadenitis-of-parotid-gland
#10
Chiyyarath Gopalan Muralidharan, Rohit Sharma, Lalit Janjani
No abstract text is available yet for this article.
August 30, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28062146/intensive-statin-regimens-for-reducing-risk-of-cardiovascular-diseases-among-human-immunodeficiency-virus-infected-population-a-nation-wide-longitudinal-cohort-study-2000-2011
#11
Huang-Tz Ou, Kai-Cheng Chang, Chung-Yi Li, Chen-Yi Yang, Nai-Ying Ko
OBJECTIVE: This study evaluated the risk of cardiovascular diseases (CVD) in a statin-treated HIV-infected population and the effects of intensive statin regimens (i.e., high-dose or potency) on CVD risks. METHODS: 945 HIV-infected patients newly on statin treatment (144, 15.7% with CVD history) were identified from Taiwan's national HIV cohort. Using the median of the first year cumulative statin dosage as a cut-off point, patients were classified into either a high-dose or low-dose group...
December 21, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28045754/anti-nxp2-positive-paraneoplastic-dermatomyositis-with-histopathologic-changes-confined-to-the-acrosyringia
#12
Jose Luis Ramírez-Bellver, Elena Macías, Claudia Bernárdez, Joaquín López-Robles, Maria Del Carmen Vegas-Sánchez, Jose Luis Díaz-Recuero, Hernán Quiceno, Luis Requena
BACKGROUND: Paraneoplastic syndromes consist of a group of disorders that are not related to the extension of the primary tumor or its metastases and that might be the first manifestation of a hidden neoplasm. It is a well-known association between dermatomyositis (DM) and cancer, especially gynecological tumors in women and lung cancer in men. METHODS: We describe the case of a 67-year-old male who developed muscular weakness and pruritic skin lesions. Skin biopsies were performed and histologic findings were consistent with DM...
January 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28044365/clinical-implications-and-hospital-outcome-of-immune-mediated-myositis-in-horses
#13
L Hunyadi, E A Sundman, P H Kass, D C Williams, M Aleman
BACKGROUND: Immune-mediated myositis (IMM) is a cause of rhabdomyolysis, stiffness, and muscle atrophy predominantly affecting Quarter horses. Limited information is available with regard to outcome, prognostic indicators, and associations with concurrent diseases. HYPOTHESIS/OBJECTIVES: To report outcomes and associations between outcome and clinical and laboratory parameters, and presence of concurrent illness. ANIMALS: Sixty-eight horses; 52 Quarter horses and related breeds and 16 other breeds...
January 2, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28043224/haycocknema-perplexum-an-emerging-cause-of-parasitic-myositis-in-australia
#14
Luke J Vos, Thomas Robertson, Enzo Binotto
Haycocknema perplexum is a rare cause of parasitic myositis, with all cases of human infection reported from Australia. This case involved an 80-year-old Queensland wildlife carer, who presented with muscle weakness, mild eosinophilia and creatine kinase elevation. This case supports an association with native animal contact and highlights the debilitating nature of this infection.
December 24, 2016: Communicable Diseases Intelligence Quarterly Report
https://www.readbyqxmd.com/read/28042944/comparison-of-serum-raav-serotype-specific-antibodies-in-patients-with-duchenne-muscular-dystrophy-becker-muscular-dystrophy-inclusion-body-myositis-or-gne-myopathy
#15
Deborah Zygmunt, Kelly E Crowe, Kevin Flanigan, Paul T Martin
Recombinant Adeno-associated virus (rAAV) is a commonly used gene therapy vector for the delivery of therapeutic transgenes in a variety of human diseases, but pre-existing serum antibodies to viral capsid proteins can greatly inhibit rAAV transduction of tissues. We have assayed serum from patients with Duchenne Muscular Dystrophy (DMD), Becker Muscular Dystrophy (DMD), Inclusion Body Myositis (IBM), and GNE myopathy (GNE). These were compared to serum from otherwise normal human subjects to determine the extent of pre-existing serum antibodies to rAAVrh74, rAAV1, rAAV2, rAAV6, rAAV8 and rAAV9...
January 2, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28039312/sarcoplasmic-mxa-expression-a-valuable-marker-of-dermatomyositis
#16
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/28034602/contribution-of-secreted-nadase-and-streptolysin-o-to-the-pathogenesis-of-epidemic-serotype-m1-streptococcus-pyogenes-infections
#17
Luchang Zhu, Randall J Olsen, Jessica D Lee, Adeline R Porter, Frank R DeLeo, James M Musser
Streptococcus pyogenes secretes many toxins that facilitate human colonization, invasion, and dissemination. NADase (SPN) and streptolysin O (SLO) are two toxins that play important roles in pathogenesis. We previously showed that increased production of SPN and SLO in epidemic serotype M1 and M89 S. pyogenes strains is associated with rapid intercontinental spread and enhanced virulence. The biological functions of SPN and SLO have been extensively studied using eukaryotic cell lines, but the relative contribution of each of these two toxins to pathogenesis of epidemic M1 or M89 strains remains unexplored...
December 27, 2016: American Journal of Pathology
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
#18
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/28012697/the-host-defense-peptide-ll-37-a-possible-inducer-of-the-type-i-interferon-system-in-patients-with-polymyositis-and-dermatomyositis
#19
Xin Lu, Quan Tang, Monica Lindh, Maryam Dastmalchi, Helene Alexanderson, Karin Popovic Silwerfeldt, Birgitta Agerberth, Ingrid E Lundberg, Cecilia Wick
The type I interferon (IFN) system has recently been suggested to play important and essential roles in the pathogenesis of myositis. However, a clarification of how type I IFNs could function as triggering factor(s) in the pathogenesis of myositis has yet failed. Through activation of the type I IFN system, the host defense peptide LL-37 carries numerous immunomodulatory properties and is implicated in the pathogenesis of several other autoimmune diseases, including systemic lupus erythematosus (SLE). The expression of LL-37 can be regulated by various endogenous factors including the active form of vitamin D (25(OH)D3)...
December 22, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28012595/orbital-autoimmune-inflammatory-disorders-protein-regional-variability-might-explain-specific-lesion-location
#20
Margo S Clarke, Alexandre Plouznikoff, Jean Deschenes
In ophthalmology, inflammatory diseases target different highly specific regions within the small confine of the orbit. Some entities even prefer a particular location or depth within the same tissue (ex. anterior, intermediate or posterior uveitides, chorioretinitides with unique topographic presentations). Though the location of a lesion strongly influences and helps us in our differential diagnosis, we still don't understand why specific anatomic sites are susceptible to a disease while other areas are spared...
January 2017: Medical Hypotheses
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