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Clin Ter 2019 Jan-Feb;170(1):e55-e60

Department of Clinical and Experimental Medicine, University of Pisa, Italy.

The chemokine monokine induced by interferon (IFN)-γ (MIG) is expressed in idiopathic inflammatory myopathies muscle. Abundant expression of MIG was observed on macrophages and T cells surrounding and invading non-necrotic muscle fibers in polymyositis and in inclusion-body myositis and in T cells in perimysial infiltrates of dermatomyositis. MIG is also localized to blood vessel endothelial cells in all inflammatory and normal muscle tissues and it exerts its biological effects mainly via binding to the chemokine (C-X-C motif) receptor (CXCR)3. Read More

Ann Rheum Dis 2019 Feb 13. Epub 2019 Feb 13.

The National Institute for Health Research Manchester Musculoskeletal Biomedical Research Centre, Manchester University Hospitals NHS FoundationTrust, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.

Objectives: With the tools available currently, confirming the diagnosis of inclusion body myositis (IBM) can be difficult. Many patients are initially misdiagnosed with polymyositis (PM). In this observational study at a UK adult neuromuscular centre, we investigated whether amyloid positron emission tomography could differentiate between IBM and PM. Read More

Lab Invest 2019 Feb 11. Epub 2019 Feb 11.

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

Inclusion body myositis (IBM) is the most common cause of primary myopathy in individuals aged 50 years and over, and is pathologically characterized by protein aggregates of p62 and mislocalized cytoplasmic TDP-43, as well as mitochondrial abnormalities in affected muscle fibers. Our recent studies have shown the accumulation of TDP-43 in mitochondria in neurons from patients with amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD), and revealed mitochondria as critical mediators of TDP-43 neurotoxicity. In this study, we investigated the association between mitochondria and TDP-43 in biopsied skeletal muscle samples from IBM patients. Read More

Nucl Med Commun 2019 Jan 18. Epub 2019 Jan 18.

Nuclear Medicine Department, Lady Davis Institute, Jewish General Hospital.

Background: To compare the performance of fluorine-18-fluorodeoxyglucose (F-FDG) PET/computed tomography (CT) and conventional tests for cancer screening in autoimmune inflammatory myopathy (AIM) patients.

Patients And Methods: We carried out a retrospective cohort study of AIM patients from one academic center in Montreal, Canada, classified using myositis-specific antibodies, who underwent F-FDG PET/CT between April 2005 and February 2018 and were followed up on average 3.5±2. Read More

Gerontology 2019 Jan 14:1-13. Epub 2019 Jan 14.

Department of Exercise Biochemistry, Korea National Sport University, Seoul, Republic of

Background: Mitochondrial dysfunction is implicated in the pathogenesis of multiple muscular diseases, including sporadic inclusion body myositis (s-IBM), the most common aging-related muscle disease. However, the factors causing mitochondrial dysfunction in s-IBM are unknown.

Objective: We hypothesized that resistance exercise (RE) may alleviate muscle impairment by improving mitochondrial function via reducing amyloid-beta (Aβ) accumulation. Read More

Autoimmun Rev 2019 Jan 11. Epub 2019 Jan 11.

Department of Microbiology and Immunology, Experimental Laboratory Immunology, KU Leuven, Leuven, Belgium; Laboratory of Medicine, University Hospitals Leuven, Leuven, Belgium. Electronic address:

Idiopathic inflammatory myopathies (IIM) are a group of diseases characterized by immune-mediated muscular lesions that may be associated with extra-muscular manifestations involving skin, lungs, heart or joints. Four main groups of IIM can be distinguished: dermatomyositis (DM), overlap myositis including mainly anti-synthetase syndrome (ASS), immune mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Myositis-specific autoantibodies (MSA) are increasingly recognized as valuable tools for diagnosis, classification and prognosis of IIM. Read More

Muscle Nerve 2019 Jan 12. Epub 2019 Jan 12.

Department of Neurology, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.

Introduction: Inclusion body myositis (IBM) can have clinical and electrodiagnostic features similar to other neuromuscular diseases, making it a diagnostic challenge. This prospective study was designed to determine the accuracy of forearm ultrasound for IBM.

Methods: Sixty adults were recruited (15 with IBM, 15 with amyotrophic lateral sclerosis [ALS], 15 with other myopathies, and 15 healthy controls), and each underwent ultrasound of the bilateral forearms (imaging the flexor digitorum profundus and flexor carpi ulnaris muscles). Read More

Biomolecules 2019 Jan 7;9(1). Epub 2019 Jan 7.

Neuromuscular Reference Centre, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium.

The sporadic form of inclusion body myositis (IBM) is the most common late-onset myopathy. Its complex pathogenesis includes degenerative, inflammatory and mitochondrial aspects. However, which of those mechanisms are cause and which effect, as well as their interrelations, remain partly obscured to this day. Read More

Curr Treatm Opt Rheumatol 2018 15;4(4):299-315. Epub 2018 Sep 15.

3Division of Rheumatology, Department of Medicine, Karolinska Institutet and Karolinska University Hospital, Solna, SE-171 76 Stockholm, Sweden.

Purpose Of Review: The purpose of this review was to give an update on treatment modalities for patients with idiopathic inflammatory myopathies, or shortly myositis, excluding the subgroup inclusion body myositis, based on a literature survey on therapies used in myositis. Few controlled trials have been performed in patients with myositis; therefore, we also included a summary of open-label trials, case series, and case reports.

Recent Findings: Glucocorticoid (GC) in high doses is still the first-line treatment of patients with myositis. Read More

Curr Treatm Opt Rheumatol 2018 23;4(4):289-298. Epub 2018 Nov 23.

Department of NVS, Huddinge, Karolinska Institutet and Department of Medicine, Karolinska Institutet, Solna and Function Area Occupational Therapy and Physical Therapy, Karolinska University Hospital, SE-171 76 Stockholm, Sweden.

Purpose Of The Review: A growing body of evidence supports exercise as a very important part of the treatment for adult patients with idiopathic inflammatory myopathies (IIM). This review mainly focuses on exercise studies published during the last 2 years in adult myositis.

Recent Findings: During the last couple of years, new publications present further evidence for intensive endurance exercise as an anti-inflammatory treatment inducing muscle growth and improving mitochondrial function compared a non-exercising control group. Read More

Cardiol Rev 2018 Dec 21. Epub 2018 Dec 21.

Idiopathic inflammatory myopathies are a group of autoimmune diseases that are characterized by muscle inflammation resulting in elevated muscle enzyme release and distinctive biopsy findings. This group of conditions includes polymyositis, dermatomyositis, inclusion body myositis and necrotizing autoimmune myopathy. Although they have many similarities, the inflammatory myopathies differ in their clinical, pathological and treatment realms. Read More

Neuromuscul Disord 2019 Jan 9;29(1):39-47. Epub 2018 Nov 9.

Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address:

Granulomatous myopathies are etiologically heterogeneous myopathies, pathologically characterized by the presence of intramuscular granulomas. Treatment outcomes are variable. We aimed to identify prognostic factors of treatment outcomes in myopathies featuring non-caseating granulomas. Read More

Methods Mol Biol 2019 ;1901:89-94

Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.

Sporadic inclusion body myositis (sIBM) is a chronic and progressive inflammatory myopathy that is the commonest among population over 50s. Recently, autoantibodies against cytosolic 5'-nucleotidase 1A (cN1A) have been identified in plasma and serum samples from patients with sIBM. So far, various methods have been established to detect the anti-cN1A autoantibodies, which showed a clinical utility of detection of the autoantibodies in the diagnosis of sIBM. Read More

Rheumatol Int 2018 Dec 8. Epub 2018 Dec 8.

Division of Histology and Embryology, Department of Human Morphology and Embryology, Wroclaw Medical University, Chałubińskiego Street 6a, 50-368, Wrocław, Poland.

Sporadic inclusion body myositis (s-IBM) is a progressive, skeletal muscle disease with poor prognosis. However, establishing the final diagnosis is difficult because of the lack of clear biomarkers in the blood serum and very slow development of clinical symptoms. Moreover, most other organs function normally without any disturbance. Read More

Med Sci (Paris) 2018 Nov 12;34 Hors série n°2:35-38. Epub 2018 Nov 12.

Inserm U955 Team 10, Paris Est-Créteil University, Créteil, France.

Dysimmune and inflammatory myopathies (DIMs) affect around 14/100,000 people worldwide. Based on immupour nopathological criteria, DIMs are divided in four groups: (1) polymyositis (PM)/inclusion body myositis (IBM), (2) dermatomyositis (DM), (3) immune-mediated necrotizing myopathies (IMNM) and (iv) overlapping myositis including anti-synthetase syndrome (ASS). ASS and PM/IBM are characterized by the activation of inflammation with lymphocytic infiltrations. Read More

EMBO Mol Med 2018 Dec;10(12)

Research Programs Unit, Molecular Neurology, Biomedicum-Helsinki, University of Helsinki, Helsinki, Finland

Mitochondrial disorders (MDs) are inherited multi-organ diseases with variable phenotypes. Inclusion body myositis (IBM), a sporadic inflammatory muscle disease, also shows mitochondrial dysfunction. We investigated whether primary and secondary MDs modify metabolism to reveal pathogenic pathways and biomarkers. Read More

Front Neurol 2018 11;9:846. Epub 2018 Oct 11.

Department of Neurology and Neuromuscular Reference Center, Ghent University Hospital, Ghent, Belgium.

We recently identified osmolyte accumulators as novel biomarkers for chronic skeletal muscle inflammation and weakness, but their precise involvement in inflammatory myopathies remains elusive. In the current study, we demonstrate that, in myoblasts and myotubes exposed to pro-inflammatory cytokines or increased salt concentration, mRNA levels of the osmolyte carriers SLC5A3, SLC6A6, SLC6A12, and AKR1B1 enzyme can be upregulated. Induction of SLC6A12 and AKR1B1 was confirmed at the protein level using immunofluorescence and Western blotting. Read More

Acta Neurol Scand 2019 Feb 8;139(2):177-182. Epub 2018 Nov 8.

Neuromuscular Centre, Department of Neurology, Sahlgrenska University Hospital, Gothenburg, Sweden.

Objectives: Inclusion body myositis is characterized by inflammatory and degenerative changes, but the temporal relation of these events is unknown.

Materials And Methods: In nineteen patients with inclusion body myositis, muscle strength was correlated with inflammatory and degenerative findings on magnetic resonance imaging (MRI) and in muscle biopsies in three different muscles (tibialis anterior, vastus lateralis, and biceps brachii). Muscle strength, measured with a handheld dynamometer, was described as percentage of muscle strength in age- and sex-matched normal individuals. Read More

Neurotherapeutics 2018 10;15(4):976-994

Department of Neurology, Brigham and Women's Hospital and Harvard Medical School, 60 Fenwood Road, Boston, MA, 02115, USA.

The major forms of autoimmune myopathies include dermatomyositis (DM), polymyositis (PM), myositis associated with antisynthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). While each of these conditions has unique clinical and histopathological features, they all share an immune-mediated component. These conditions can occur in isolation or can be associated with systemic malignancies or connective tissue disorders (overlap syndromes). Read More

Ther Adv Musculoskelet Dis 2018 Oct 7;10(10):201-207. Epub 2018 Oct 7.

Octapharma France, Boulogne-Billancourt, France.

Immunoglobulin (Ig) therapy is used to treat a wide range of immunodeficiencies and autoimmune diseases; While, its clinical benefit has been demonstrated in several studies, Ig therapy is associated with a risk of systemic adverse effects. As such, Onset of renal impairment, including acute renal failure, osmotic nephrosis and renal insufficiency, after immunoglobulin administration is rare, but is one of the most significant concerns related to intravenous Ig use at immunomodulatory doses. However, only few studies have investigated the safety of subcutaneous Ig (SCIg) in relation to these rare conditions. Read More

Neurology 2018 Oct;91(16):760

From the Hôpital Pitié-Salpêtrière (O.L.-C., O.B.), AP-HP, Department of Internal Medicine and Clinical Immunology, Inflammation-Immunopathology-Biotherapy Department (I2B), East Paris Neuromuscular Diseases Reference Center, Inserm U974, France; Departments of Kinesiology and Surgery (F.P.), University of Montreal, B-Temia Inc. (S.B.), Québec City, and Division of Rheumatology (M.H.), Department of Medicine, Jewish General Hospital, Lady Davis Institute, Montréal, Québec, Canada.

Dtsch Med Wochenschr 2018 10 4;143(20):1472-1476. Epub 2018 Oct 4.

Ehem. Ärztlicher Leiter der Rheumaklinik und des Rheumaforschungsinstituts Aachen.

Idiopathic inflammatory myopathies (IIM) are a rare and clinically polymorphic and heterogenous group of myositis syndromes. Myositis is part of a systemic autoimmune disorder with various extramuscular manifestations affecting skin, lungs, joints, esophagus and other organ systems. Most myositis patients have autoantibodies against non organspecific antigens. Read More

J Am Acad Orthop Surg 2018 Oct 2. Epub 2018 Oct 2.

From the Department of Orthopaedic Surgery, University of Alabama at Birmingham Hospital, Birmingham, AL.

Introduction: Orthopaedic surgeons are wary of patients with neuromuscular (NM) diseases as a result of perceived poor outcomes and lack of data regarding complication risks. We determined the prevalence of patients with NM disease undergoing total joint arthroplasty (TJA) and characterized its relationship with in-hospital complications, prolonged length of stay, and total charges.

Methods: Data from the Nationwide Inpatient Sample from 2005 to 2014 was used for this retrospective cohort study to identify 8,028,435 discharges with total joint arthroplasty. Read More

Neuropathol Appl Neurobiol 2018 Sep 28. Epub 2018 Sep 28.

Department of Internal Medicine and Clinical Immunology, Paris-Sorbonne University, Public Assistance-Hospitals of Paris (APHP), Pitié-Salpêtrière University Hospital, INSERM, UMR974, Inflammation-Immunopathology-Biotherapy Department (DHU I2B), and Reference Center for Neuromuscular Pathologies, Institute of Myology, Paris, France.

Aims: To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analyzing different DM subforms, and to test the superiority of MxA to other markers.

Methods: Immunohistochemistry for MxA and retinoic acid-inducible gene I (RIG-I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti-Mi-2 (n=6), -TIF1-γ (n=10), -NXP2 (n=13), -MDA5 (n=10), or -SAE (n=1) autoantibodies and with no detectable autoantibody (n=17). Among the patients, 9 suffered from cancer and 22 were juvenile-onset type. Read More

Int J Neurosci 2018 Nov 26:1-6. Epub 2018 Nov 26.

c Third Department of Neurology , G. Papanikolaou General Hospital , Thessaloniki , Greece.

Background: Sporadic inclusion body myositis is the most common inflammatory myopathy over the age of 50. The aetiopathogenesis of the disease remains unclear and to the day there is no effective treatment.

Objectives: The aim of the present review is to present the latest data on the new insights and developments in the treatment of sporadic inclusion body myositis, focusing on Bimagrumab and Alemtuzumab. Read More

Am J Med Case Rep 2018 23;6(8):157-160. Epub 2018 Aug 23.

Divisions of Cardiovascular Disease and Rheumatology, Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn, New York, U.S.A- 11203.

Idiopathic inflammatory myopathy (IIM) is a rare autoimmune myopathy that includes polymyositis, dermatomyositis, inclusion body myositis and autoimmune necrotizing myositis. Cardiac involvement was considered a rare occurrence in IIM however, recent reports suggests that cardiac involvement is a common feature and portends poor prognosis as it is usually encountered in advanced disease. IIM leads to myocarditis with subsequent development of myocardial fibrosis, cardiac conduction system disease and cardiomyopathy resulting in both systolic and diastolic heart failure. Read More

JAMA Neurol 2018 Dec;75(12):1528-1537

Centre de Recherche en Myologie, Unité Mixte de Recherche Scientifique 974, Université Pierre et Marie Curie, Institut National de la Santé et de la Recherche Médicale, Paris, France.

Importance: Idiopathic inflammatory myopathies are heterogeneous in their pathophysiologic features and prognosis. The emergence of myositis-specific autoantibodies suggests that subgroups of patients exist.

Objective: To develop a new classification scheme for idiopathic inflammatory myopathies based on phenotypic, biological, and immunologic criteria. Read More

Neurotherapeutics 2018 10;15(4):995-1005

Neuromuscular Medicine Division, Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, 66103, USA.

Inclusion body myositis is the most common acquired myopathy after the age of 50. It is characterized by progressive asymmetric weakness predominantly affecting the quadriceps and/or finger flexors. Loss of ambulation and dysphagia are major complications of the disease. Read More

Exp Neurol 2018 Nov 18;309:169-180. Epub 2018 Aug 18.

Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan.

Muscle histology of sporadic inclusion body myositis (sIBM) demonstrates inflammatory findings and degenerative features including accumulation of TAR DNA-binding protein of 43 kDa (TDP-43). However, whether sarcoplasmic accumulation of TDP-43 is a primary trigger of muscle degeneration or a secondary event resulting from muscle degeneration in the pathophysiology of sIBM remained unclear. Our study aimed to discover whether muscle-dominant expression of TDP-43 is a primary cause of muscle degeneration. Read More

Lancet Neurol 2018 Sep;17(9):816-828

Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Inflammatory myopathies, collectively known as myositis, are heterogeneous disorders characterised by muscle inflammation, and frequently accompanied by extramuscular manifestations that affect the skin, lung, and joints. Patients with inflammatory myopathies were previously classified as having dermatomyositis if characteristic rashes accompanied the muscle involvement, and as having polymyositis if no rashes were present. Five main types of inflammatory myopathies are now widely recognised: dermatomyositis, immune-mediated necrotising myopathy, sporadic inclusion-body myositis, overlap myositis (including antisynthetase syndrome), and polymyositis. Read More

RMD Open 2018 25;4(2):e000661. Epub 2018 Jul 25.

Department of Rheumatology, University Hospitals Leuven, Leuven, Belgium.

Objective: As rheumatoid factor (RF) and anticitrullinated protein antibodies (ACPAs) are not routinely tested in idiopathic inflammatory myositis (IIM), little is known about their prevalence and clinical implications in this patient group. In antisynthetase syndrome (ASS), presence of ACPA is reportedly associated with more severe and erosive arthritis. We aim to retrospectively determine the prevalence of RF and ACPA in a cross-sectional cohort of 121 patients diagnosed with IIM and to assess clinical associations. Read More

Biochem Biophys Res Commun 2018 09 9;503(4):2770-2777. Epub 2018 Aug 9.

Institute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nuremberg, 91054, Erlangen, Germany. Electronic address:

Heterozygous missense mutations in the human VCP gene cause inclusion body myopathy associated with Paget disease of bone and fronto-temporal dementia (IBMPFD) and amyotrophic lateral sclerosis (ALS). The exact molecular mechanisms by which VCP mutations cause disease manifestation in different tissues are incompletely understood. In the present study, we report the comprehensive analysis of a newly generated R155C VCP knock-in mouse model, which expresses the ortholog of the second most frequently occurring human pathogenic VCP mutation. Read More

Prog Lipid Res 2018 10 9;72:1-17. Epub 2018 Aug 9.

Orthopaedic Research & Biotechnology, The Children's Hospital at Westmead, Westmead, NSW, Australia.; Discipline of Paediatrics & Child Heath, Faculty of Medicine, University of Sydney, Camperdown, NSW, Australia. Electronic address:

Lipid storage myopathies (LSMs) are a heterogeneous group of genetic disorders that present with abnormal lipid storage in multiple body organs, typically muscle. Patients can clinically present with cardiomyopathy, skeletal muscle weakness, myalgia, and extreme fatigue. An early diagnosis is crucial, as some LSMs can be managed by simple nutraceutical supplementation. Read More

Neuromuscul Disord 2018 Sep 27;28(9):778-786. Epub 2018 Jun 27.

Division of Genetics and Genomic Medicine, Department of Pediatrics, University of California, Irvine Medical Center, 101 The City Drive South, ZC4482, Orange, CA 92868, United States. Electronic address:

Inclusion body myopathy (IBM) associated with Paget disease of the bone and frontotemporal dementia or IBMPFD is an autosomal dominant degenerative disorder caused by mutations in the valosin-containing protein (VCP) gene. We aim to establish a detailed clinical phenotype of VCP disease amongst 35 (28 affected individuals, 7 presymptomatic gene carriers) individuals versus 14 unaffected first-degree relatives in 14 families to establish useful biomarkers for IBMPFD and identify the most meaningful tests for monitoring disease progression in future clinical trials. Comprehensive studies included the Inclusion Body Myositis Functional Rating Scale (IBMFRS) and fatigue severity scale questionairres, strength measurements using the Manual Muscle Test with Medical Research Council (MRC) scales, hand-held dynamometry using the microFET and Biodex dynamometers, 6 minute walk test (6MWT), and pulmonary function studies. Read More

Adv Clin Exp Med 2018 Oct;27(10):1453-1457

Department of Histology and Embryology, Faculty of Medicine, Wroclaw Medical University, Poland.

Sporadic inclusion body myositis (sIBM) is a rare yet increasingly prevalent disease and the most common cause of inflammatory myopathy in people over the age of 50. The exact cause of the disorder is unknown. In sIBM 2 processes, first autoimmune and the other degenerative, parallelly occur in the muscle cells. Read More

Curr Opin Rheumatol 2018 Nov;30(6):644-649

Faculty of Biology Medicine and Health, Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester.

Purpose Of Review: To describe recent advancements in diagnostic and therapeutic approaches to inclusion body myositis (IBM).

Recent Findings: Our understanding of the implications of anti-cytosolic 5'-nucleotidase 1A autoantibody status in IBM and other diseases is increasing. Muscle imaging using magnetic resonance techniques and ultrasound is increasingly being performed and characteristic patterns of muscle involvement may help with diagnosis. Read More

Curr Rheumatol Rep 2018 Aug 3;20(9):56. Epub 2018 Aug 3.

UPMC Univ Paris 06, INSERM UMRS_974, Center of Research in Myology, AP-HP, Department of Internal Medicine & Clinical Immunology, DHU I2B, Pitié-Salpêtrière Hospital, Sorbonne Université, F-75013, Paris, France.

Purpose Of Review: This review provides an overview of the potential pathogenic roles of anti-SRP and anti-HMGCR in IMNM over the past 5 years.

Recent Findings: Idiopathic inflammatory myopathies (IIM) are a group of acquired autoimmune disorders that mainly affect the skeletal muscle tissue. Classification criteria of IIM are comprised of polymyositis, dermatomyositis, inclusion body myositis and immune-mediated necrotizing myopathies. Read More

Neurol India 2018 Jul-Aug;66(4):1062-1066

Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

Aim: To study C4d expression as a marker of complement activation in the diagnosis of dermatomyositis.

Material And Methods: Muscle biopsies from patients diagnosed as definite dermatomyositis (10), nonspecific myositis associated with connective tissue disease (9), necrotizing autoimmune myositis (1), inclusion body myositis (1), and normal muscle (1) according to European Neuromuscular criteria 2004 were studied for C4d expression and capillary loss on CD 34 immunohistochemistry.

Results: C4d was expressed in all biopsies of definite dermatomyositis in the perimysial vessels and in 3/10 endomysial capillaries corresponding to capillary loss on CD 34. Read More

Curr Treat Options Neurol 2018 Jul 23;20(9):37. Epub 2018 Jul 23.

Department of Neurology, University of California, Irvine, CA, USA.

Purpose Of Review: The purpose of this review is to update the audience on the recent progress in the treatment of idiopathic inflammatory myopathies, highlighting a myriad of treatment trials aimed at slowing down progression of muscle weakness and/or skin involvement in idiopathic inflammatory myopathies.

Recent Findings: Myositis continues to be an active area of clinical and translational research. Through the work of a number of investigators, we now have a much better understanding of the pathogenesis underlying the various myositides. Read More

Muscle Nerve 2019 Jan 18;59(1):70-75. Epub 2018 Dec 18.

Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Introduction: It is unclear whether quantitating muscle endurance adds nonredundant information useful for the care of patients with muscular disease.

Methods: Records were retrospectively reviewed for all Johns Hopkins Myositis Center patients with a muscle endurance assessment (n = 128, 226 patient-visits). Muscle endurance and strength were quantitated with the Myositis Functional Index-2 (FI2) and manual muscle testing (MMT), respectively. Read More

Muscle Nerve 2018 Oct;58(4):E25-E26

Department of Neurology, Duke School of Medicine, Durham, North Carolina, USA.

Neuromuscul Disord 2018 Aug 18;28(8):660-664. Epub 2018 Jun 18.

Department of Research, Hospital for Special Care, New Britain, CT, USA.

Sporadic inclusion body myositis (IBM) is the most common acquired myopathy affecting patients over age 50. The discovery of an autoantibody directed against a 43-44 kD protein (anti-cytosolic-5'-nucleotidase 1A or anti-cN1A) has provided support for the hypothesis of an immune-mediated pathogenesis. Previous studies have reported variable test sensitivity and specificity, and inconsistent results on the predictive value. Read More

Front Neurol 2018 26;9:456. Epub 2018 Jun 26.

MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, London, United Kingdom.

Distal myopathies are a diagnostically challenging group of diseases. We wanted to understand the value of MRI in the current clinical setting and explore the potential for optimizing its clinical application. We retrospectively audited the diagnostic workup in a distal myopathy patient cohort, reassessing the diagnosis, whilst documenting the usage of MRI. Read More

Clin Rheumatol 2018 Aug 3;37(8):2113-2123. Epub 2018 Jul 3.

Division of Rheumatology, University of California Los Angeles, Los Angeles, CA, USA.

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. Read More

Curr Rheumatol Rep 2018 Jun 28;20(8):52. Epub 2018 Jun 28.

Neuromuscular Division, Department of Neurology and the Institute for Neurological Discoveries, The University of Kansas Medical Center, 3901 Rainbow Blvd, Mail Stop 2012, Kansas City, KS, 66160, USA.

Purpose Of Review: While sporadic inclusion body myositis (sIBM) is the most common acquired muscle disease after age 50, the pathogenesis of this disease is still poorly understood. In this review, we discuss our current state of knowledge in sIBM and provide an update on our current understanding of its pathophysiology and management.

Recent Findings: Lines of evidence in support of an inflammatory pathogenesis include inflammatory infiltrates in the target organ, NFκB activation, cytokine response, MHC I upregulation, and cN1A antibody. Read More

Front Immunol 2018 5;9:1200. Epub 2018 Jun 5.

Department of Biomolecular Chemistry, Radboud Institute for Molecular Life Sciences and Institute for Molecules and Materials, Radboud University, Nijmegen, Netherlands.

Introduction: Autoantibodies to cytosolic 5'-nucleotidase 1A (cN-1A; NT5C1A) have a high specificity when differentiating sporadic inclusion body myositis from polymyositis and dermatomyositis. In primary Sjögren's syndrome (pSS) and systemic lupus erythematosus (SLE) anti-cN-1A autoantibodies can be detected as well. However, various frequencies of anti-cN-1A reactivity have been reported in SLE and pSS, which may at least in part be explained by the different assays used. Read More

Nucleus 2018 ;9(1):398-409

a Neurology IV - Neuroimmunology and Neuromuscular Diseases Unit , Fondazione IRCCS Istituto Neurologico "Carlo Besta" , Milan , Italy.

Laminopathies are a heterogeneous group of diseases, caused by mutations in lamin A/C proteins. The most common laminopathy (LMNA-related myopathies, LMNA-RM) affects skeletal and cardiac muscles; muscle histopathology is variable, ranging from mild unspecific changes to dystrophic features, sometimes with inflammatory evidence. Whether the genetic defect might activate innate immune components, leading to chronic inflammation, myofiber necrosis and fibrosis, is still unknown. Read More

Autoimmun Rev 2018 Aug 6;17(8):746-754. Epub 2018 Jun 6.

Department of Neurology, Ghent University and Ghent University Hospital, C. Heymanslaan 10, 9000 Gent, Belgium.

Dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), immune mediated necrotizing myopathy (IMNM) and overlap myositis (OM) are classified as inflammatory myopathies (IM) with involvement of autoimmune features such as autoreactive lymphocytes and autoantibodies. Autoimmunity can be defined as a loss in self-tolerance and attack of autoantigens by the immune system. Self-tolerance is achieved by a group of immune mechanisms occurring in central and periphal lymphoid organs and tissues, called immune checkpoints, that work in synergy to protect the body from harmful immune reactions. Read More

Curr Med Res Opin 2018 Sep 5;34(9):1679-1685. Epub 2018 Jul 5.

c Novartis Pharmaceuticals Corporation , New York , NY , USA.

Objective: This study identifies the health care costs and utilization, as well as comorbidities, in a Medicare population of inclusion body myositis (IBM) patients.

Methods: Medicare patients aged ≥65 years with a diagnosis claim for IBM were identified and matched to a cohort of non-IBM patients based on age, sex, race, calendar year and census region. Generalized linear models were used to estimate health care costs and utilization during the follow-up period. Read More