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idiopathic inflammatory myopathy

John Svensson, Ingrid E Lundberg, Mia Von Euler, Elizabeth V Arkema, Marie Holmqvist
OBJECTIVE: To study the occurrence of ischemic (IS) and haemorrhagic stroke (HS) in idiopathic inflammatory myopathies (IIM) compared to the general population and to investigate how it varies by sex, age, clinical sub-diagnosis and time since IIM diagnosis. METHODS: All newly diagnosed IIM patients in Sweden were identified from the National Patient Register and general population comparators were identified from the Total Population Register. The study population was followed prospectively until death, emigration, December 2013 or first incident stroke...
July 12, 2018: Arthritis Care & Research
Lina Su, Yue Yang, Yuan Jia, Xu Liu, Wei Zhang, Yun Yuan, Zhanguo Li
RATIONALE: Idiopathic inflammatory myopathies have been extensively reported associated with malignancy. Immune-mediated necrotizing myopathy (IMNM), however, has rarely been connected with malignancy including acute myeloid leukemia (AML). PATIENT CONCERNS: A 65-year-old woman was diagnosed with AML and received regular chemotherapy. After the 5th cycle chemotherapy, she achieved complete remission but developed severe muscle weakness and myalgia with dramatic increasing creatine kinase (CK)...
July 2018: Medicine (Baltimore)
Siming Gao, Xiaoxia Zuo, Di Liu, Yizhi Xiao, Honglin Zhu, Huali Zhang, Hui Luo
BACKGROUND: Dermatomyositis and polymyositis are the best known idiopathic inflammatory myopathies (IIMs). Classic histopathologic findings include the infiltration of inflammatory cells into muscle tissues. Neutrophil serine proteinases (NSPs) are granule-associated enzymes and play roles in inflammatory cell migration by increasing the permeability of vascular endothelial cells. In this study, we aimed to find the roles of NSPs in pathogenesis of IIMs. METHODS: RNA and DNA were isolated to measure the relative expression of NSPs and their methylation levels...
July 5, 2018: Arthritis Research & Therapy
S Barsotti, E Cioffi, A Tripoli, A Tavoni, A D'Ascanio, M Mosca, R Neri
Rituximab (RTX), a chimeric monoclonal antibody targeted against CD20, has been used to treat refractory inflammatory myopathies (IIM). The primary objective of this study was to retrospectively assess the efficacy of RTX in reducing disease activity in patients with IIM refractory to conventional therapy. Secondary aim was the evaluation of adverse events (AE) during the treatment period. We examined 26 patients with a diagnosis of IIM, referred to our Rheumatology Unit and treated with RTX for active refractory disease...
July 6, 2018: Reumatismo
Sangmee Bae, Christina Charles-Schoeman
To describe the use of oral cyclophosphamide (PO CYC) in a single center longitudinal cohort of patients with idiopathic inflammatory myopathies (IIM). Patients using PO CYC were identified through a retrospective chart review of a myositis cohort at a single academic center. PO CYC dose, duration, adverse events, and disease activity measures before and after CYC were analyzed. Disease activity measures included muscle enzymes, manual muscle testing (MMT8), 100-mm visual analog scale (VAS), and 1-4 Likert scale for physician global assessment...
July 3, 2018: Clinical Rheumatology
Nicolò Pipitone, Carlo Salvarani
The idiopathic inflammatory myopathies (IIM) dermatomyositis (DM) and polymyositis (PM) are chronic diseases affecting the striated muscles with variable involvement of other organs. Glucocorticoids are considered the cornerstone of treatment, but some patients require adjunctive immunosuppressive agents because of insufficient response to glucocorticoids, flares upon glucocorticoid tapering, or glucocorticoid-related adverse events. Areas covered: The aim of this article was to review (PubMed search until February 2018) the evidence on established and new therapies derived from randomized controlled trials (RCTs) on adult DM and PM...
July 3, 2018: Expert Review of Clinical Immunology
E E Sigmund, S H Baete, T Luo, K Patel, D Wang, I Rossi, A Duarte, M Bruno, D Mossa, A Femia, S Ramachandran, D Stoffel, J S Babb, A G Franks, J Bencardino
INTRODUCTION: Dermatomyositis (DM) is an idiopathic inflammatory myopathy involving severe debilitation in need of diagnostics. We evaluated the proximal lower extremity musculature with diffusion tensor imaging (DTI), intravoxel incoherent motion (IVIM) and dynamic DTI in DM patients and controls and compared with standard clinical workup.  METHODS: In this IRB-approved, HIPAA-compliant study with written informed consent, anatomical, Dixon fat/water and diffusion imaging were collected in bilateral thigh MRI of 22 controls and 27 DM patients in a 3T scanner...
June 4, 2018: European Radiology
Richard D Sontheimer
It is now widely accepted that long-term aminoquinoline antimalarial therapy with hydroxychloroquine (HCQ) can mitigate one of the most important comorbidities of systemic lupus erythematosus (LE)-atherosclerotic cardiovascular disease (ASCVD). Increasing evidence suggests that idiopathic inflammatory myopathy (IIM) patients have a risk for ASCVD comorbidity that is similar to that of systemic LE. I would like to explore the primary hypothesis that long-term HCQ therapy could provide those with IIM, especially dermatomyositis (DM) patients, an ASCVD comorbidity benefit similar to that of systemic LE...
April 2018: Annals of Translational Medicine
Lisa K Peterson, Troy D Jaskowski, Sonia L La'ulu, Anne E Tebo
The aim of this study was to examine the frequency and significance of antibodies targeting the small ubiquitin-like modifier 1 activating enzyme (SAE) in patients under serologic evaluation for idiopathic inflammatory myopathies. Patient sera (n = 17) recognizing bands at approximately 40 (SAE1) and 90 (SAE2) kDa were identified in 6445 consecutive samples for myositis autoantibody evaluation by immunoprecipitation (IP) of S35 -labeled K562 cell lysate. All 17 positive samples, 176 disease, and 67 healthy controls were evaluated for SAE1 antibodies using a line immunoblot assay (LIA)...
June 2018: Immunologic Research
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
Naoki Mugii, Fujiko Someya
Idiopathic inflammatory myopathies involve skeletal muscles and can be associated with interstitial lung disease and/or heart dysfunction, which may reduce exercise capacity. We aimed to clarify cardiopulmonary factors affecting the 6-min walk distance in patients who were able to walk without leg pain or fatigue. Twenty-three patients with inactive adult idiopathic inflammatory myopathies, and 18 age- and gender-matched healthy controls were evaluated for hemodynamic responses using noninvasive impedance cardiography during the 6-min walk test...
May 14, 2018: Rheumatology International
Leandro Ladislau, Débora M Portilho, Tristan Courau, Alhondra Solares-Pérez, Elisa Negroni, Jeanne Lainé, David Klatzmann, Adriana Bonomo, Yves Allenbach, Olivier Benveniste, Ingo Riederer, Wilson Savino, Vincent Mouly, Gillian Butler-Browne, Claudia F Benjamim
Idiopathic Inflammatory Myopathies (IIMs) are a heterogeneous group of autoimmune diseases affecting skeletal muscle tissue homeostasis. They are characterized by muscle weakness and inflammatory infiltration with tissue damage. Amongst the cells in the muscle inflammatory infiltration, dendritic cells (DCs) are potent antigen-presenting and key components in autoimmunity exhibiting an increased activation in inflamed tissues. Since, the IIMs are characterized by the focal necrosis/regeneration and muscle atrophy, we hypothesized that DCs may play a role in these processes...
May 10, 2018: Cell Death & Disease
Sandra Pérez-Baos, Iván Prieto-Potin, Jorge A Román-Blas, Olga Sánchez-Pernaute, Raquel Largo, Gabriel Herrero-Beaumont
Besides its primary function in locomotion, skeletal muscle (SKM), which represents up to half of human's weight, also plays a fundamental homeostatic role. Through the secretion of soluble peptides, or myokines, SKM interacts with major organs involved in metabolic processes. In turn, metabolic cues from these organs are received by muscle cells, which adapt their response accordingly. This is done through an intricate intracellular signaling network characterized by the cross-talking between anabolic and catabolic pathways...
2018: Frontiers in Physiology
Sobiya Mahnaz Ayesha, A K Meena, Navatha Vangala, Liza Rajasekhar, Subhash Kaul, Rupam Borgahain, Megha S Uppin
Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic, autoimmune disorders which include a new entity, necrotizing autoimmune myopathy (NAM). NAM lacks inflammation and presents with markedly elevated creatinine phosphokinase (CPK) levels. It is associated with connective tissue diseases (CTDs), statin use, malignancies, and most cases are idiopathic. Objectives: The objectives of this study are to describe the clinicopathologic features in muscle biopsy-proven cases of NAM...
January 2018: Annals of Indian Academy of Neurology
Paula A O Araujo, Marilda Guimarães Silva, Eduardo Ferreira Borba, Samuel K Shinjo
OBJECTIVES: A high frequency of metabolic syndrome (MetS) has been recently described in different idiopathic inflammatory myopathies, but not in antisynthetase syndrome (ASS). Therefore, the aim of the present study was to determine the prevalence of MetS in ASS and also its possible association with cardiovascular the risk factors and ASS-related disease characteristics. METHODS: A cross-sectional single centre study of 42 consecutive ASS patients was conducted from 2012 to 2015 and compared to 84 healthy individuals matched for gender, age, ethnicity and body mass index-matched (control group)...
March 2018: Clinical and Experimental Rheumatology
G Keyßer, S Zierz, M Kornhuber
OBJECTIVES: To gain information about the efficacy of immunosuppressive drugs as first-, second-, and third-line treatment of idiopathic inflammatory myopathies (IIM). METHODS: 112 treatment cycles of 63 patients with dermatomyositis (n = 23), polymyositis (n = 33), overlap syndromes (n = 4), and undifferentiated connective tissue diseases (n = 3) were analyzed by retrospective chart analysis. Data regarding muscle strength, muscle enzymes, treatment duration, and treatment discontinuation were collected...
April 30, 2018: Zeitschrift Für Rheumatologie
Frederick W Miller, Janine A Lamb, Jens Schmidt, Kanneboyina Nagaraju
Autoimmune diseases develop as a result of chronic inflammation owing to interactions between genes and the environment. However, the mechanisms by which autoimmune diseases evolve remain poorly understood. Newly discovered risk factors and pathogenic processes in the various idiopathic inflammatory myopathy (IIM) phenotypes (known collectively as myositis) have illuminated innovative approaches for understanding these diseases. The HLA 8.1 ancestral haplotype is a key risk factor for major IIM phenotypes in some populations, and several genetic variants associated with other autoimmune diseases have been identified as IIM risk factors...
April 20, 2018: Nature Reviews. Rheumatology
Neil J McHugh, Sarah L Tansley
The discovery of novel autoantigen systems related to idiopathic inflammatory myopathies (collectively referred to as myositis) in adults and children has had major implications for the diagnosis and management of this group of diseases across a wide range of medical specialties. Traditionally, autoantibodies found in patients with myositis are described as being myositis-specific autoantibodies (MSAs) or myositis-associated autoantibodies (MAAs), depending on their prevalence in other, related conditions. However, certain MSAs are more closely associated with extramuscular manifestations, such as skin and lung disease, than with myositis itself...
April 20, 2018: Nature Reviews. Rheumatology
Heather O Tory, Ruy Carrasco, Thomas Griffin, Adam M Huber, Philip Kahn, Angela Byun Robinson, David Zurakowski, Susan Kim
BACKGROUND: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. METHODS: Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA...
April 19, 2018: Pediatric Rheumatology Online Journal
Hirokazu Sasaki, Hitoshi Kohsaka
Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are major IIM subsets. Immune-mediated necrotizing myopathy became recognized as a potentially new IIM subset. Since the new classification criteria published by the International Myositis Classification Criteria Project have higher sensitivity and specificity for IIM classification and subclassification than the previous criteria, they should help precise diagnosis...
May 9, 2018: Modern Rheumatology
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