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Inflammatory myopathy

Sainan Bian, Hua Chen, Li Wang, Yunyun Fei, Yunjiao Yang, Linyi Peng, Yongzhe Li, Fengchun Zhang
Patients with primary biliary cholangitis (PBC) can have extrahepatic manifestations. However, data about cardiac involvement of PBC is limited. We aimed in this study to analyze the clinical characteristics in patients with PBC complicated with and without cardiac involvement, and the risk factors of cardiac involvement in PBC. PBC patients admitted to Peking Union Medical College Hospital between January 2002 and February 2016 were consecutively enrolled. Structured interview, systemic rheumatologic examination, and laboratory tests were conducted for each patient, and risk factors of cardiac involvement were analyzed by comparing patients with and without cardiac involvement...
2018: PloS One
Ya-Mei Zhang, Han-Bo Yang, Jing-Li Shi, He Chen, Xiao-Ming Shu, Xin Lu, Guo-Chun Wang, Qing-Lin Peng
Autoantibodies against poly-U-binding factor 60 kDa protein (PUF60) have been reported in Caucasian dermatomyositis (DM) patients. However, their clinical significance in idiopathic inflammatory myopathy (IIM) remains to be fully clarified. Our objective was to analyze the prevalence and clinical significance of anti-PUF60 antibodies in a large cohort of Chinese IIM patients. In our study, 388 IIM patients, 301 disease controls, and 167 healthy controls (HCs) were involved. An enzyme-linked immunosorbent assay (ELISA) was developed to detect serum anti-PUF60 levels and was validated using immunoblotting methods...
March 15, 2018: Clinical Rheumatology
Boaz Palterer, Gianfranco Vitiello, Alessia Carraresi, Maria Grazia Giudizi, Daniele Cammelli, Paola Parronchi
Idiopathic inflammatory myopathies represent a heterogeneous group of autoimmune diseases with systemic involvement. Even though numerous specific autoantibodies have been recognized, they have not been included, with the only exception of anti-Jo-1, into the 2017 Classification Criteria, thus perpetuating a clinical-serologic gap. The lack of homogeneous grouping based on the antibody profile deeply impacts the diagnostic approach, therapeutic choices and prognostic stratification of these patients. This review is intended to highlight the comprehensive scenario regarding myositis-related autoantibodies, from the molecular characterization and biological significance to target antigens, from the detection tools, with a special focus on immunofluorescence patterns on HEp-2 cells, to their relative prevalence and ethnic diversity, from the clinical presentation to prognosis...
2018: Clinical and Molecular Allergy: CMA
James B Lilleker, Axel C P Diederichsen, Søren Jacobsen, Mark Guy, Mark E Roberts, Jamie C Sergeant, Robert G Cooper, Louise P Diederichsen, Hector Chinoy
Objectives: Limitations in the methods available for identifying cardiac involvement and accurately quantifying disease activity in the idiopathic inflammatory myopathies (IIMs) may contribute to poor outcomes. We investigated the utility of different serum muscle damage markers [total creatine kinase (CK), cardiac troponin T (cTnT) and cardiac troponin I (cTnI)] to address these issues. Methods: We assessed disease activity and cardiac involvement using the International Myositis Assessment and Clinical Studies Group core set measures in 123 participants with confirmed adult-onset IIM from the UK and Denmark...
March 12, 2018: Rheumatology
Hanbo Yang, Xin Lu, Qinglin Peng, Wei Jiang, Jingli Shi, Yamei Zhang, He Chen, Guochun Wang
OBJECTIVE: To investigate the associations between anti-NXP-2 autoantibody levels and disease activity as well as calcinosis severity. METHODS: The serum levels of anti-NXP-2 autoantibodies were determined in 709 idiopathic inflammatory myopathies (IIMs) and also serially measured by an in-house enzyme-linked immunosorbent assay using recombinant MORC3. Patients with anti-NXP-2 autoantibodies were divided into two subgroups: with or without calcinosis. The associations of anti-NXP-2 autoantibody levels with organ-specific disease activity, serum creatine kinase (CK) levels, and calcinosis severity were investigated in cross-sectional and longitudinal analyses...
March 13, 2018: Arthritis & Rheumatology
Masahiro Ueki, Ichiro Kobayashi, Shunichiro Takezaki, Yusuke Tozawa, Yuka Okura, Masafumi Yamada, Masataka Kuwana, Tadashi Ariga
OBJECTIVES: The aim of our study is to clarify the association of myositis-specific autoantibodies (MSAs) with clinical and laboratory features in Japanese patients with juvenile idiopathic inflammatory myopathies (JIIMs). METHODS: We retrospectively analyzed the frequency of MSAs and their association with clinical or laboratory findings in 25 Japanese patients with JIIMs in Hokkaido district. RESULTS: Eighteen of 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), 5 with anti-transcriptional intermediary factor (TIF)-1γ (20%), 4 with anti-MJ/nuclear matrix protein (NXP)-2 (16%), 2 with anti-Jo-1 (8%), 1 with anti- HMG-CoA reductase, 1 with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each...
March 13, 2018: Modern Rheumatology
Ali Maisam Afzali, Thomas Müntefering, Heinz Wiendl, Sven G Meuth, Tobias Ruck
Histopathological analyses of muscle specimens from myositis patients indicate that skeletal muscle cells play an active role in the interaction with immune cells. Research over the last few decades has shown that skeletal muscle cells exhibit immunobiological properties that perfectly define them as non-professional antigen presenting cells. They are able to present antigens via major histocompatibility complex molecules, exhibit costimulatory molecules and secrete soluble molecules that actively shape the immune response in an either pro- or anti-inflammatory manner...
March 8, 2018: Autoimmunity Reviews
Jolien Claessens, Thibaut Belmondo, Ellen De Langhe, Rene Westhovens, Koen Poesen, Sophie Hüe, Daniel Blockmans, Michael Mahler, Marvin J Fritzler, Xavier Bossuyt
Solid phase assays (SPAs) and automated microscope systems are increasingly used to screen for antinuclear antibodies (ANAs). The goal of this study was to evaluate the performance of three automated ANA screening assays; NOVA Lite HEp-2 using NOVA View® (NV, Inova Diagnostics), an automated indirect immunofluorescence method, EliA™ CTD Screen (Fluorescence Enzyme Immunoassay, FEIA; Thermo Fisher) and QUANTA Flash® CTD Screen Plus (Chemiluminescence immunoassay, CIA; Inova Diagnostics). The assays were performed on 480 diagnostic samples from patients with an ANA-associated rheumatic disease (AARD; systemic lupus erythematosus, primary Sjögren's syndrome, systemic sclerosis, inflammatory myopathy, mixed connective tissue disease) and on 767 samples from diseased and healthy controls...
March 8, 2018: Autoimmunity Reviews
Airi Nishimi, Takeo Isozaki, Shinichiro Nishimi, Sho Ishii, Takahiro Tokunaga, Hidekazu Furuya, Kuninobu Wakabayashi, Tsuyoshi Kasama
The original version of this article, unfortunately, contained errors. Figure citation, caption, image and updated sentence in the Result section are now presented correctly in this article.
March 10, 2018: Clinical Rheumatology
Angel Santos Briz, Anaid Calle, Konstantinos Linos, Bruce Semans, Andrew Carlson, Omar P Sangüeza, Dieter Metze, Lorenzo Cerroni, José Luis Díaz-Recuero, Victoria Alegría-Landa, José Manuel Mascaró, Carmen Moreno, Jose Luis Rodríguez-Peralto, Luis Requena
BACKGROUND: Panniculitis occurring in dermatomyositis is uncommon, with only a few cases described in the literature, most of them as case reports. OBJECTIVE: This report describes the clinico-pathological and immunohistochemical findings in a series of 18 patients with panniculitis associated to dermatomyositis. METHODS: In each patient we collected the clinical data of the cutaneous lesions as well as the characteristic clinical and laboratory findings...
March 10, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Ying Hou, Yue-Bei Luo, Tingjun Dai, Kai Shao, Wei Li, Yuying Zhao, Jian-Qiang Lu, Chuanzhu Yan
The European Neuromuscular Centre (ENMC) pathological classification criteria of idiopathic inflammatory myopathies (IIMs) are debatable. The aim of this study was to explore their practicability and reproducibility. We conducted a retrospective analysis of 57 cases of IIMs excluding dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) by in-depth analysis of muscle biopsies and comparisons of the clinical characteristics among polymyositis (PM), non-specific myositis (NSM) and necrotizing autoimmune myopathy (NAM)...
March 7, 2018: Journal of Neuropathology and Experimental Neurology
Paul M Rossman, Stephanie A Thomovsky, Ryan M Schafbuch, Ling T Guo, G D Shelton
A 2-year-old male, intact Boxer was referred for chronic diarrhea, hyporexia, labored breathing, weakness and elevated creatine kinase, and alanine aminotransferase activities. Initial examination and diagnostics revealed a peripheral nervous system neurolocalization, atrial premature complexes, and generalized megaesophagus. Progressive worsening of the dog's condition was noted after 36 h; the dog developed aspiration pneumonia, was febrile and oxygen dependent. The owners elected humane euthanasia. Immediately postmortem biopsies of the left cranial tibial and triceps muscles and the left peroneal nerve were obtained...
2018: Frontiers in Veterinary Science
Anna Tjärnlund, Matteo Bottai, Ingrid E Lundberg
We have with great interest read the letter titled "Comments on the "2017 EULAR/ACR Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups". Points of concern", by Dr Castañeda et al published in your journal [1]. The authors discuss the antisynthetase syndrome (ASSD), a condition characterized by myositis, arthritis, interstitial lung disease (ILD), Raynaud's phenomenon and the presence of autoantibodies targeting aminoacyl transfer RNA synthetases, and the fact that this group was not included in the 2017 EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups [2]...
March 7, 2018: Arthritis & Rheumatology
J Lim, A van Royen-Kerkhof, R E Jonkers, M V Starink, A E Voskuyl, A J van der Kooi
Idiopathic inflammatory myopathy (IIM), commonly referred to as "myositis", is a rare but treatable auto-immune disease that is often misdiagnosed or diagnosed after significant delay. Using three clinical case reports as introductory examples, an overview is given - and pitfalls are discussed - of the diagnosis and treatment of myositis. Disease features are often extra-muscular in nature, may vary considerably between patients, and are frequently non-specific. Myositis-related morbidity is high and myositis can be fatal, mainly due to cancer and interstitial lung disease...
2018: Nederlands Tijdschrift Voor Geneeskunde
M M Wennekers, M Appelman, W F van der Weele
BACKGROUND: Dermatomyositis is an idiopathic inflammatory myopathy with an incidence of 1 in 100,000. Clinically, it features typical skin abnormalities and muscle weakness. In a subset of cases an underlying malignancy may be present; in such instances the term paraneoplastic syndrome is used. CASE DESCRIPTION: We saw a 75-year-old male with progressive skin abnormalities, especially on the chest and hands. In addition, he experienced weakness in his arms and legs...
2018: Nederlands Tijdschrift Voor Geneeskunde
S G Gofrit, H Yonath, M Lidar, Y Shoenfeld, S Kivity
Inflammatory myopathies are a clinically diverse group of diseases, in which the detection of particular autoantibodies may facilitate diagnosis, treatment, and prognosis. The aim of this report is to summarize our experience with specific autoantibody testing in patients with inflammatory myopathies. Data were collected over the last decade in the Autoimmune Center of the Sheba Medical Center, a tertiary referral hospital. Data regarding patients' positive for autoantibodies against Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, and PM-Scl antigens were retrospectively collected...
February 16, 2018: Clinical Rheumatology
Edward Smitaman, Dyan V Flores, Catalina Mejía Gómez, Mini N Pathria
Atraumatic disorders of skeletal muscles include congenital variants; inherited myopathies; acquired inflammatory, infectious, or ischemic disorders; neoplastic diseases; and conditions leading to muscle atrophy. These have overlapping appearances at magnetic resonance (MR) imaging and are challenging for the radiologist to differentiate. The authors organize muscle disorders into four MR imaging patterns: (a) abnormal anatomy with normal signal intensity, (b) edema/inflammation, (c) mass, and (d) atrophy, highlighting each of their key clinical and imaging findings...
February 16, 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Esther Barreiro
Skeletal muscle weakness is common in the intensive care units (ICU). Approximately 50% of patients under mechanical ventilation for more than 7 days show signs of ICU-acquired muscle weakness. In these patients, muscle weakness may be the result of axonal polyneuropathy, myopathy or a combination of both. The commonest risk factors in patients with ICU-acquired weakness (AW) are the severity and duration of the systemic inflammatory response, duration of the stay in the ICU and of mechanical ventilation, hyperglycemia, hypoalbuminemia, parenteral nutrition, and administration of corticosteroids and of neuromuscular blocking agents...
January 2018: Annals of Translational Medicine
Adrian T Huber, Marine Bravetti, Jérôme Lamy, Tania Bacoyannis, Charles Roux, Alain de Cesare, Aude Rigolet, Olivier Benveniste, Yves Allenbach, Mathieu Kerneis, Philippe Cluzel, Nadjia Kachenoura, Alban Redheuil
BACKGROUND: Idiopathic inflammatory myopathy (IIM) is a group of autoimmune diseases with systemic myositis which may involve the myocardium. Cardiac involvement in IIM, although often subclinical, may mimic clinical manifestations of acute viral myocarditis (AVM). Our aim was to investigate the usefulness of the combined analysis of cardiovascular magnetic resonance (CMR) T1 and T2 mapping parameters measured both in the myocardium and in the thoracic skeletal muscles to differentiate AVM from IIM cardiac involvement...
February 12, 2018: Journal of Cardiovascular Magnetic Resonance
B Debashree, Manish Kumar, T S Keshava Prasad, Archana Natarajan, Rita Christopher, A Nalini, P S Bindu, N Gayathri, M M Srinivas Bharath
Mitochondria regulate the balance between lipid metabolism and storage in the skeletal muscle. Altered lipid transport, metabolism and storage influence the bioenergetics, redox status and insulin signalling contributing to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neurological disorders which entail intramuscular lipid accumulation and impaired mitochondrial bioenergetics in the skeletal muscle causing progressive myopathy with muscle weakness. However, the mitochondrial changes including molecular events associated with impaired lipid storage have not been completely understood in the human skeletal muscle...
February 9, 2018: Journal of Neurochemistry
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