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Inflammatory myositis

Angela Ceribelli, Natasa Isailovic, Maria De Santis, Elena Generali, Micaela Fredi, Ilaria Cavazzana, Franco Franceschini, Luca Cantarini, Minoru Satoh, Carlo Selmi
This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis...
October 20, 2016: Clinical Rheumatology
Robert M Kwee, Shivani Ahlawat, Jan Fritz
OBJECTIVE: To determine the frequency of magnetic resonance imaging (MRI) findings of ischiofemoral impingement (IFI) in patients with inflammatory myositis (IM) and associated factors. METHODS: Pelvis and thigh MRI studies of 314 consecutive patients (57% women; mean age, 55.5 years; range, 18-85) with suspected muscle disease were reviewed. RESULTS: Ischiofemoral impingement at MRI was present in 11% of patients with a final diagnosis of IM and in 2% of patients with an alternative diagnosis (P = 0...
October 18, 2016: Journal of Computer Assisted Tomography
Thomas Schwartz, Louise Pyndt Diederichsen, Ingrid E Lundberg, Ivar Sjaastad, Helga Sanner
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention...
2016: RMD Open
Chang-Fu Kuo, Shue-Fen Luo, Kuang-Hui Yu, Lai-Chu See, Weiya Zhang, Michael Doherty
BACKGROUND: Systemic sclerosis (SSc) is a rare and devastating disease affecting skin and internal organs. Familial aggregation of SSc and co-aggregation with other autoimmune diseases is rarely reported. METHODS: We identified 23,658,577 beneficiaries registered with the National Health Insurance database in 2010, 1891 of whom had SSc. We identified 21,009,551 parent-child relationships and 17,168,340 full sibling pairs. The familial risks of SSc and other autoimmune diseases and familial transmission were estimated...
October 12, 2016: Arthritis Research & Therapy
Sreejith V Ravi, M Raveendran, C J Selvakumar, M Sacratis, T Geetha
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Sreejith V Ravi, M Raveendran, C J Selvakumar, M Sacratis, T Geetha
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
P Devic, L Gallay, N Streichenberger, P Petiot
Amongst the heterogeneous group of inflammatory myopathies, focal myositis (FM) stands as a rare and benign dysimmune disease. Although FM can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of FM is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern...
August 26, 2016: Neuromuscular Disorders: NMD
Saleh Saleh Elessawy, Eman Muhammad Abdelsalam, Eman Abdel Razek, Samar Tharwat
BACKGROUND: Conventional magnetic resonance imaging (MRI) is a highly valuable tool for full assessment of the extent of bilateral symmetrical diffuse inflammatory myopathy, owing to its high sensitivity in the detection of edema which correlates with, and sometimes precedes, clinical findings. PURPOSE: To evaluate the use of whole-body (WB)-MRI in characterization and full assessment of the extent and distribution of diffuse inflammatory myopathy. MATERIAL AND METHODS: A prospective study on 15 patients presenting with clinical evidence of inflammatory myopathy...
September 2016: Acta Radiologica Open
Andrea Váncsa
Using current recommended treatment, a majority of patients with idiopathic inflammatory myopathy develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis, as well as in active disease and inclusion body myositis to some extent. Importantly, randomized controlled trials indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity...
September 2016: Orvosi Hetilap
Matthew B Carroll, Michelle R Newkirk, Nathan S Sumner
Necrotizing autoimmune myopathy (NAM) is a recently recognized entity within the spectrum of idiopathic inflammatory myopathies. Diagnosis critically rests on histopathologic demonstration of macrophage predominant myocyte destruction, with few to no lymphocytes. We report our experience with identifying and treating this subset of inflammatory myositis, highlighting the importance of muscle biopsy in diagnosis, association with statin use and malignancy, and challenges of therapy.We present 3 cases that presented to 2 hospitals within our academic system in calendar year 2014 with acute/subacute onset of profound proximal muscle weakness and markedly elevated creatine kinase levels...
October 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Iago Pinal-Fernandez, Maria Casal-Dominguez, John A Carrino, Arash H Lahouti, Pari Basharat, Jemima Albayda, Julie J Paik, Shivani Ahlawat, Sonye K Danoff, Thomas E Lloyd, Andrew L Mammen, Lisa Christopher-Stine
OBJECTIVES: The aims of this study were to define the pattern of muscle involvement in patients with immune-mediated necrotising myopathy (IMNM) relative to those with other inflammatory myopathies and to compare patients with IMNM with different autoantibodies. METHODS: All Johns Hopkins Myositis Longitudinal Cohort subjects with a thigh MRI (tMRI) who fulfilled criteria for IMNM, dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) or clinically amyopathic DM (CADM) were included in the study...
September 20, 2016: Annals of the Rheumatic Diseases
Tomoko Noda, Masahiro Iijima, Seiya Noda, Shinya Maeshima, Hirotaka Nakanishi, Seigo Kimura, Haruki Koike, Shinsuke Ishigaki, Yohei Iguchi, Masahisa Katsuno, Gen Sobue
Objective An association has been reported between inflammatory myopathies (IMs), which include polymyositis (PM) and dermatomyositis (DM), and malignancy, and the concept of cancer-associated myositis (CAM) was recently proposed. We herein attempted to determine the features and etiologies of these myopathies. Methods We analyzed the gene expression levels via microarray and real-time quantitative reverse transcription polymerase chain reaction analyses to identify genes that were specifically upregulated or downregulated with suspected inflammatory involvement and verified the microarray data via an immunohistochemical (IHC) analysis in additional cases...
2016: Internal Medicine
Thomas Khoo, Sophia Otto, Caroline Smith, Barbara Koszyca, Sue Lester, Peter Blumbergs, Vidya Limaye
The clinical significance of curvilinear bodies (CB) seen in association with hydroxychloroquine (HCQ) therapy is uncertain. Patients with CB on muscle biopsy performed between 2006 and the present were identified, and their clinical features including body mass index and cumulative HCQ dose were recorded. A control group of 16 patients with idiopathic inflammatory myositis (IIM) on HCQ at time of biopsy but without evidence of CB was identified. Nineteen patients with CB were identified; details were available for 18...
September 14, 2016: Clinical Rheumatology
Alexandra C Braun, Marcus Gutmann, Regina Ebert, Franz Jakob, Henning Gieseler, Tessa Lühmann, Lorenz Meinel
PURPOSE: The inhibition of myostatin - a member of the transforming growth factor (TGF-β) family - drives regeneration of functional skeletal muscle tissue. We developed a bioresponsive drug delivery system (DDS) linking release of a myostatin inhibitor (MI) to inflammatory flares of myositis to provide self-regulated MI concentration gradients within tissues of need. METHODS: A protease cleavable linker (PCL) - responding to MMP upregulation - is attached to the MI and site-specifically immobilized on microparticle surfaces...
September 14, 2016: Pharmaceutical Research
L Gallay, P Petiot
Sporadic inclusion-body myositis (sIBM) is the most frequent myopathy after 50 years of age. As the clinical presentation may often be typical, pathological confirmation by muscle biopsy appears necessary, but sometimes difficult. Further delineation of the framework of this particular disease, especially during its early-onset stage, appears to be challenging. New classification of diagnostic criteria as well as the identification of new diagnostic hallmarks appear to be the two main tools towards to achieve this purpose...
October 2016: Revue Neurologique
Laura Nuño, Beatriz Joven, Patricia Carreira, Valentina Maldonado, Carmen Larena, Irene Llorente, Eva Tomero, María Carmen Barbadillo, Paloma García-de la Peña, Lucía Ruiz, Juan Carlos López-Robledillo, Henry Moruno, Ana Pérez, Tatiana Cobo-Ibáñez, Raquel Almodóvar, Leticia Lojo, Indalecio Monteagudo, María Jesús García-De Yébenes, Francisco Javier López-Longo
OBJECTIVE: To analyze clinical characteristics, survival and causes of death of patients diagnosed with autoimmune inflammatory myositis in the REMICAM registry from the Society of Rheumatology in the Community of Madrid (SORCOM). METHODS: Multicenter cohort of patients diagnosed with autoimmune inflammatory myopathy with follow-up between January 1980 and December 2014. A total of 313 variables concerning demographic, clinical and morbidity data were collected, and a comparison was performed between clinical subgroups...
September 5, 2016: Reumatología Clinica
S Almeida, M Braga, M J Santos
Serologic investigation has been explored in inflammatory myopathies in order to define subgroups that can help us predict clinical course, treatment and prognosis. The level of similarity between juvenile and adult myopathies regarding the presence of myositis-specific autoantibodies has not been fully elucidated. We report the case of a 8-year-old girl who presented with a rapid progression of muscle weakness and cutaneous signs consistent with the diagnosis of juvenile dermatomyositis and whose serologic testing revealed the presence of anti-signal recognition particle (SRP) antibodies...
April 2016: Acta Reumatológica Portuguesa
Leah J Witt, James J Curran, Mary E Strek
Anti-synthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease, myositis, Raynaud's phenomenon, and arthritis. There is a higher prevalence and increased severity of interstitial lung disease in patients with anti-synthetase syndrome, as compared to dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. Diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results...
September 2016: Clinical Pulmonary Medicine
Rahul Sehgal, Rafael Medina-Flores, Ralph Yachoui, Charles Kenney
Immune mediated necrotizing myopathy (IMNM) is a unique form of myositis that is characterized by distinct muscle biopsy features including abundant myofiber necrosis, degeneration, and regeneration with only minimal, if any, inflammation on muscle biopsy. IMNM is clinically similar to idiopathic inflammatory myopathy (IIM); hence, muscle biopsy is essential to diagnose IMNM. Herein we describe a case of neck extensor weakness due to necrotizing myopathy. Isolated weakness of the neck extensor muscles is uncommon in IIM and IMNM...
August 29, 2016: Clinical Medicine & Research
Adam P Lightfoot, Robert G Cooper
PURPOSE OF REVIEW: This article updates on the concept that muscle-derived cytokines (myokines) play important roles in muscle health and disease. RECENT FINDINGS: Interleukin-6 (IL-6) is released from normal skeletal muscle in response to exercise, mediating both anti-inflammatory responses and metabolic adaptations, actions contradictory to the prevailing view that IL-6 is a proinflammatory cytokine that is inducing and propagating disease. The anti-inflammatory effects of IL-6 result from its trans-membrane signalling capability, via membrane-bound receptors, whereas its proinflammatory effects result instead from signalling via the soluble IL-6 receptor and gp130...
November 2016: Current Opinion in Rheumatology
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