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Dtsch Med Wochenschr 2020 Jul 2;145(13):903-910. Epub 2020 Jul 2.

Myositis is a rare and an extremely heterogeneous autoimmune disease, that causes muscle weakness. Currently, "idiopathic inflammatory myopathies (IIM)" is the preferred umbrella-term used to describe the disease complexity within individuals. IIM include dermatomyositis, polymyositis, inclusion body myositis, autoimmune necrotizing myopathy, overlap myositis and antisynthetase syndrome. Read More

Rheumatol Int 2020 Jun 27. Epub 2020 Jun 27.

German Rheumatism Research Centre, Epidemiology Unit, Charitéplatz 1, 10117, Berlin, Germany.

Objective: To describe trends in outcomes among patients with idiopathic inflammatory myopathies (IIM) over two decades.

Methods: From 1997 to 2017, a total of 1079 IIM patients were documented in the National Database of the German Collaborative Arthritis Centers. Annual cross-sectional data on treatment, disease activity, patient-reported outcomes, hospitalization and employment were compared across the years. Read More

Front Neurol 2020 10;11:513. Epub 2020 Jun 10.

Assistant Professor of Neurology, Department of Neurology, University of Missouri, Columbia, MO, United States.

Drooling related to bulbar weakness and dysfunction is a common concern in patients with neuromuscular disease. While there are numerous medications to manage sialorrhea, they are often limited by side effects and lack of efficacy. Botulinum toxin has shown to benefit ALS patients in a few studies, but there is scant data on the benefit in other neuromuscular conditions. Read More

J Neurol Neurosurg Psychiatry 2020 Jun 23. Epub 2020 Jun 23.

Department of Neurology, Amsterdam Neuroscience, Amsterdam UMC, Amsterdam, Noord-Holland, The Netherlands.

Objectives: To describe the combination of spinocerebellar ataxia (SCA) types 3 and 6 and sporadic inclusion body myositis (IBM).

Methods: A description of five patients with SCA type 3 and 6 who were diagnosed with IBM. We explore possible mechanisms explaining the coexistence of both diseases. Read More

Ann Rheum Dis 2020 Jun 16. Epub 2020 Jun 16.

Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Insititutes of Health, Bethesda, Maryland, USA

Objectives: Myositis is a heterogeneous family of diseases that includes dermatomyositis (DM), antisynthetase syndrome (AS), immune-mediated necrotising myopathy (IMNM), inclusion body myositis (IBM), polymyositis and overlap myositis. Additional subtypes of myositis can be defined by the presence of myositis-specific autoantibodies (MSAs). The purpose of this study was to define unique gene expression profiles in muscle biopsies from patients with MSA-positive DM, AS and IMNM as well as IBM. Read More

BMC Rheumatol 2020 10;4:25. Epub 2020 Jun 10.

Centre for Epidemiology, Division of Population Health, Health Services Research & Primary Care, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, University of Manchester, Manchester, UK.

Background: The idiopathic inflammatory myopathies (IIMs) are heterogeneous autoimmune conditions of skeletal muscle inflammation and weakness. MicroRNAs (miRNAs) are short, non-coding RNA which regulate gene expression of target mRNAs. The aim of this study was to profile miRNA and mRNA in IIM and identify miRNA-mRNA relationships which may be relevant to disease. Read More

Muscle Nerve 2020 Jun 8. Epub 2020 Jun 8.

Division of Neuromuscular Medicine, Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Background: Limited data exist regarding myopathies with early or prominent dysphagia.

Methods: A retrospective study was performed (January 2003 to August 2019) to identify myopathy patients in whom dysphagia was the initial symptom or was disproportionately severe compared with limb weakness.

Results: Thirty-two patients were identified. Read More

Muscle Nerve 2020 Jun 1. Epub 2020 Jun 1.

Department of Neurology, Mayo Clinic, 200 1st Street SW, Rochester, Minnesota, 55905, USA.

Muscle disorders are characterized by differential involvement of various muscle groups. Among these, weakness predominantly affecting finger flexors is an uncommon pattern, most frequently found in sporadic inclusion-body myositis. This finding is particularly significant when the full range of histopathological findings of inclusion-body myositis is not found on muscle biopsy. Read More

J Clin Neuromuscul Dis 2020 Jun;21(4):246-247

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

J Clin Med 2020 May 13;9(5). Epub 2020 May 13.

Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona, 08036 Barcelona, Spain.

Sporadic inclusion body myositis (sIBM) is an inflammatory myopathy associated, among others, with mitochondrial dysfunction. Similar molecular features are found in Alzheimer's disease (AD) and Type 2 Diabetes Mellitus (T2DM), underlying potential comorbidity. This study aims to evaluate common clinical and molecular hallmarks among sIBM, AD, and T2DM. Read More

Int J Neurosci 2020 May 4:1-10. Epub 2020 May 4.

Third Department of Neurology, Aristotle University of Thessaloniki, Greece.

Sporadic inclusion body myositis is the most common idiopathic inflammatory myopathy over the age of 50, with a male-to-female ratio of 3:1. Symptoms onset before age of 60 occurs in 18-20% of patients, with a delay in diagnosis of 5 to 8 years.The classic clinical presentation of SIBM consists of proximal leg and distal arm weakness, and most commonly patients present early slowly progressive quadriceps weakness which leads to falls and to difficulties in climbing stairs, while less common the initial complaints refer to finger flexor weakness and atrophy, foot drop, or dysphagia, and rare presentations include prominent forearm weakness, sparing the quadriceps. Read More

Front Cell Dev Biol 2020 17;8:226. Epub 2020 Apr 17.

Experimental Therapeutics Laboratory, University of South Australia Cancer Research Institute, Adelaide, SA, Australia.

Introduction: High Mobility Group Box Protein 1 (HMGB1) is a DNA-binding protein that exerts inflammatory or pro-repair effects upon translocation from the nucleus. We postulate aberrant HMGB1 expression in immune-mediated necrotising myopathy (IMNM).

Methods: Herein, we compare HMGB1 expression (serological and sarcoplasmic) in patients with IMNM with that of other myositis subtypes using immunohistochemistry and ELISA. Read More

Dig Dis Sci 2020 Jun;65(6):1656-1660

Divisions of Gastroenterology and Hepatology, Department of Medicine, University of New Mexico School of Medicine, Albuquerque, NM, USA.

Muscle Nerve 2020 Jul 30;62(1):7-9. Epub 2020 Apr 30.

Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, Ohio, United States.

Neuromuscul Disord 2020 Apr 24;30(4):294-300. Epub 2020 Feb 24.

School of Medicine, The University of Notre Dame, Fremantle, Western Australia 6160, Australia; Department of Neurology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia; The Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, Western Australia, Australia.

Inclusion Body Myositis (IBM) is the most common acquired myopathy in older individuals with more than two thirds of patients experiencing impaired swallowing. There are currently no standardized exercise therapies to improve or sustain swallowing despite good evidence for exercise therapy in limb muscles. Reduced upper esophageal sphincter opening is a common abnormality associated with dysphagia in IBM. Read More

J Neurol Sci 2020 Jun 1;413:116816. Epub 2020 Apr 1.

Department of Medicine/Neurology, McMaster University, Hamilton, Ontario, Canada; Department of Pediatrics, McMaster University, Hamilton, Ontario, Canada.

Lipofuscin aggregation may result from incomplete degradation of damaged mitochondria by autophagy-lysosome pathway, and intra-mitochondrial lipofuscin aggregation may exacerbate mitochondrial abnormalities in mitochondrial myopathy (MM) and mitochondrial disease. We examined vastus lateralis muscle biopsies from 24 patients with pathologically diagnosed MM and clinically diagnosed chronic progressive external ophthalmoplegia, in comparison to the biopsies from 3 other groups:10 patients with inclusion body myositis (IBM), 11 younger adults, and 10 older subjects with no to minimal myopathic changes. Lipofuscin aggregation in muscle fibres was assessed on autofluorescence microscopy, some histochemical stains, and electron microscopy (EM). Read More

Scand J Immunol 2020 Jun 10;91(6):e12887. Epub 2020 May 10.

Department of Neurology, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China.

Inclusion body myositis (IBM) is a disease with a poor prognosis and limited treatment options. This study aimed at exploring gene expression profile alterations, investigating the underlying mechanisms and identifying novel targets for IBM. We analysed two microarray datasets (GSE39454 and GSE128470) derived from the Gene Expression Omnibus (GEO) database. Read More

Semin Neurol 2020 Jun 6;40(3):342-348. Epub 2020 Apr 6.

Department of Neurology, Dell Medical School, University of Texas, Austin, Texas.

The inflammatory myopathies comprise disorders of immune-mediated muscle injury. The histopathology and clinical features help distinguish them. Juvenile dermatomyositis (JDM) is the most common form of myositis in children and adolescents. Read More

Muscle Nerve 2020 Jun 11;61(6):783-788. Epub 2020 Apr 11.

School of Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland.

Introduction: The diagnosis of inclusion body myositis (IBM) can be challenging, and its presentation can be confused with other forms of myositis or neuromuscular disorders. In this study we evaluate the ability of quantitative muscle ultrasound to differentiate between IBM and mimicking diseases.

Methods: Patients 50 years of age and older were included from two specialty centers. Read More

Rinsho Shinkeigaku 2020 Apr 31;60(4):264-267. Epub 2020 Mar 31.

Department of Neurology, Kansai Electric Power Hospital.

A 71-year-old man presented with progressive muscle weakness of the four limbs in November 2014. His symptoms had started from the left leg in 2008, resulting in frequent falls. In 2011, he became unable to stand up without a handrail due to weakness of the both legs. Read More

Biomolecules 2020 Mar 30;10(4). Epub 2020 Mar 30.

Department of Neurology; Laboratory for Neuropathology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium.

Myo-inositol exerts many cellular functions, which include osmo-protection, membrane functioning, and secondary messaging. Its Na/myo-inositol co-transporter SLC5A3 is expressed in muscle tissue and further accumulates in myositis. In this study we focused on the peculiar subgroup of sporadic inclusion body myositis (IBM), in which auto-inflammatory responses and degenerative changes co-exist. Read More

Semin Arthritis Rheum 2020 Feb 29. Epub 2020 Feb 29.

Department of Internal Medicine and Clinical Immunology and Inflammation-Immunopathology-Biotherapy Department (I2B), Pitié-Salpêtrière University Hospital, Assistance Publique-Hôpitaux de Paris, East Paris Neuromuscular Diseases Reference Center, Inserm U974, Sorbonne Université, Paris 6, Paris, France.

Background: Our objective was to define the pattern and severity of muscle damage in immune-mediated necrotizing myopathy (IMNM) and its relationship with clinical and serological features.

Methods: IMNM patients with a whole-body MRI (n=42) were included and compared to sporadic inclusion-body myositis (s-IBM) patients (n=60). Fat replacement was estimated using the Mercuri score in 55 muscles. Read More

Mediterr J Rheumatol 2019 Sep 30;30(3):155-161. Epub 2019 Sep 30.

EUROIMMUN AG, Luebeck, Germany.

Myopathies are a rare type of acquired, chronic autoimmune diseases of the skeletal muscles and affect both children and adults. The hallmark symptoms of idiopathic inflammatory myopathies (IIM) are muscle inflammation, proximal muscle weakness and disability, arthritis, cutaneous rashes, calcinosis, ulceration, malignancy and interstitial lung disease (ILD). Subforms of IIM include polymyositis, dermatomyositis, cancer-related myositis and sporadic inclusion body myositis. Read More

J Neurol Sci 2020 Jun 29;413:116764. Epub 2020 Feb 29.

Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Japan.

Objective: To show the predictive risk factors for aspiration pneumonia and prognostic importance of a cricopharyngeal bar (CPB) on videofluoroscopic examination of swallowing (VFS) in inclusion body myositis (IBM).

Methods: In this retrospective study, we examined a consecutive series of 37 patients with clinico-pathologically defined IBM based on the European Neuromuscular Center diagnostic criteria for IBM from 2013. The Swallowing Disturbance Questionnaire was used for the evaluation of dysphagia. Read More

Georgian Med News 2020 Jan(298):80-83

1Petre Sarajishvili Institute of Neurology, Tbilisi, Georgia.

While it is the most common inflammatory myopathy among middle-aged and elderly people, sporadic inclusion body myositis (IBM) presents as the most challenging disease to diagnose. The prevalence of IBM varies greatly depending on geographical, ethnic and age factors. Frequency of the disease incidence among the general population ranges from 1:1,000,000 to 1:14,000. Read More

Muscle Nerve 2020 Jul 13;62(1):76-82. Epub 2020 Mar 13.

Department of Neurology, Royal Victoria Hospitals, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Queen Victoria Road, Newcastle, United Kingdom.

Introduction: Objective information on longitudinal disease progression in inclusion body myositis (IBM) is lacking.

Methods: Longitudinal dynamometry and functional status data were collated from a cohort of IBM patients. Annual change was calculated by means of linear modeling. Read More

Cureus 2020 Jan 14;12(1):e6656. Epub 2020 Jan 14.

Neurology, Flowers Medical Group, Dothan, USA.

The association between sarcoid myopathy and inclusion body myositis is a rare phenomenon that is not well understood. In this case, we present a 46-year-old female with a five-year history of sarcoidosis who became refractory to treatment, experiencing progressive deterioration and muscle wasting. The patient's distribution of muscle weakness did not follow characteristic patterns of inclusion body myositis. Read More

Best Pract Res Clin Rheumatol 2020 Feb 13;34(1):101486. Epub 2020 Feb 13.

Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK; Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK. Electronic address:

The idiopathic inflammatory myopathies are a group of heterogeneous autoimmune connective tissue diseases. Despite increase in the understanding of these conditions, securing a timely diagnosis and accurate subtype classification remains difficult in some cases. This has important implications for patients, where delayed or inappropriate treatments can have a negative effect on outcomes. Read More

Neuromuscul Disord 2020 03 17;30(3):200-206. Epub 2019 Dec 17.

Department of Neurology, University of Washington Medical Center, Box 356465, 1959 NE Pacific Street. Seattle, WA 98195-6465, United States. Electronic address:

Inclusion body myositis (IBM) is a slowly progressive and heterogeneous disorder that is a challenge for measuring clinical trial efficacy. The current methods of measuring progression of the disease utilizes the Inclusion Body Myositis Functional Rating Scale, grip strength by dynamometer, and finger flexor strength. One of the hallmarks of the disease is selective deep finger flexor weakness. Read More

Best Pract Res Clin Rheumatol 2020 Feb 8;34(1):101484. Epub 2020 Feb 8.

Johns Hopkins University School of Medicine, 5200 Eastern Avenue, MFL Building, Center Tower Suite 4500, Baltimore, MD 21224 USA. Electronic address:

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of inflammatory myopathies whose common feature is immune-mediated muscle injury. There are distinct subgroups including dermatomyositis (DM), polymyositis (PM), inclusion body myositis, and immune-mediated necrotizing myopathy. Antisynthetase syndrome is also emerging as a distinct subgroup with its unique muscle histopathological characteristic of perifascicular necrosis. Read More

Muscle Nerve 2020 05 15;61(5):570-574. Epub 2020 Feb 15.

Immunology Department, Royal Adelaide Hospital, Adelaide, South Australia, Australia.

Herein we report a case of sporadic inclusion-body myositis (sIBM) occurring at an unusually young age in a patient with primary Sjögren syndrome, and use the case to explore possible shared mechanisms for disease susceptibility. Possible factors may include the association of both conditions with the 8.1 ancestral haplotype; the presence of anti-cN1A antibodies, which, although considered specific for sIBM, are also seen in pSS; and the shared association with T-cell large granular lymphocyte leukemia (T-LGLL). Read More

Semin Arthritis Rheum 2020 06 28;50(3):492-497. Epub 2019 Dec 28.

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

Background: In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) published new classification criteria for idiopathic inflammatory myopathies (IIM).

Objectives: To [1] assess the performance of the EULAR/ACR criteria in a monocentric cohort of consecutive patients with IIM, compare them with the Bohan and Peter (BP) criteria, and with the physician's diagnosis; and [2] evaluate the effect of including the presence of interstitial lung disease (ILD) as variable in the criteria.

Methods: 439 consecutive patients with a diagnosis of IIM followed at the Rheumatology Clinic, Karolinska University Hospital, Sweden were enrolled. Read More

Front Neurol 2019 17;10:1386. Epub 2020 Jan 17.

North Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan.

The accumulation of multiple-protein aggregates within muscle fibers is a pathological hallmark of sporadic inclusion body myositis (s-IBM) with the presence of inclusion bodies. Amyloid-beta is one of the accumulated proteins in s-IBM. The aim of this study was to elucidate the utility of Pittsburgh compound B-positron emission tomography (PIB-PET) for diagnosing s-IBM. Read More

Neuromuscul Disord 2020 02 12;30(2):128-136. Epub 2019 Dec 12.

Department of Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address:

Dropped head syndrome can be the presenting feature of a wide spectrum of neurological conditions. In this study, we aimed to define the clinical characteristics and treatment outcomes of 107 patients, where head drop was the presenting or predominant clinical feature of a myopathy. Median age at presentation was 68 years (range 42-88). Read More

J Neurol 2020 May 29;267(5):1407-1413. Epub 2020 Jan 29.

Heart Clinic Ulm, Ulm, Germany.

Objective: To investigate cardiac involvement in patients with sporadic inclusion body myositis (IBM) by cardiac magnetic resonance tomography (CMR).

Methods: A case series of 20 patients with IBM underwent basic cardiac assessment and CMR including functional imaging, native and contrast-enhanced T1-weighted, and late gadolinium enhancement (LGE) imaging.

Results: All IBM patients reported no cardiac symptoms. Read More

Muscle Nerve 2020 04 8;61(4):496-503. Epub 2020 Feb 8.

Centre de référence PACA Réunion Rhône Alpes, La Timone University Hospital, Aix-Marseille University, Marseille.

Background: Fat infiltration in individual muscles of sporadic inclusion body myositis (sIBM) patients has rarely been assessed.

Methods: Sixteen sIBM patients were assessed using MRI of the thighs and lower legs (LL). The severity of fat infiltration, proximal-to-distal and side asymmetries, and the correlations with clinical and functional parameters were investigated. Read More

Ther Adv Musculoskelet Dis 2020 8;12:1759720X19886494. Epub 2020 Jan 8.

Department of Neurology, Muscle Immunobiology Group, Neuromuscular Center, University Medical Center Göttingen, Robert-Koch-Sr. 40, 37075 Göttingen, Germany.

The myositis syndromes include polymyositis, dermatomyositis (DM), necrotizing myopathy, inclusion body myositis (IBM), antisynthetase syndrome and overlap syndromes with myositis. These syndromes mostly occur in middle-aged patients, while juvenile DM occurs in children and adolescents. Patients mostly show a subacute weakness and myalgia in the upper and lower limbs, the diagnosis is based upon these clinical findings in combination with muscle biopsy results and specific serum autoantibodies. Read More

Physiol Meas 2020 02 5;41(1):015003. Epub 2020 Feb 5.

Institute of Myology, Neuromuscular Investigation Center, Pitié-Salpêtrière University Hospital, Paris, France. Author to whom any correspondence should be addressed.

Objective: Muscle strength is a critical clinical hallmark in both health and disease. The current study introduces a novel portable device prototype (MyoQuad) for assessing and monitoring maximal voluntary isometric knee extension torque (MVIT).

Approach: Fifty-six patients with inclusion body myositis were studied. Read More

Cytokine 2020 Mar 2;127:154966. Epub 2020 Jan 2.

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

Background: The cytokine growth differentiation factor-15 (GDF-15) has been associated with inflammatory and mitochondrial disease, warranting exploration of its expression in myositis patients.

Methods: GDF-15 protein levels are evaluated in 35 idiopathic inflammatory myopathy (IIM) serum samples using enzyme-linked immunosorbent assays, comparing with levels in samples from healthy individuals and from patients with genetically confirmed hereditary muscular dystrophies and mitochondrial disorders. Muscle tissue expression of GDF-15 protein is evaluated using immunofluorescent staining and Western blotting. Read More

Eur J Case Rep Intern Med 2019 12;6(12):001368. Epub 2019 Dec 12.

Centro Hospitalar do Porto, Porto, Portugal.

Inclusion body myositis (IBM) is a chronic inflammatory myopathy with a progressive course. It is more common in the later years of life and usually presents with limb weakness. We present the case of a patient who developed proximal weakness in the lower limbs and, four years later, facial asymmetry. Read More

Neurology 2020 03 27;94(9):e910-e920. Epub 2019 Dec 27.

From the Département d'Immunologie Clinique et Médecine Interne (Y.D.) and Département de Rhumatologie (J.-E.G., J.S., A.M.), Centre de Référence des Maladies Auto-immunes Rares, Département d'Immunobiologie (B.N.), Département de Pathologie (B.L.), Département de Neurologie, Centre de Référence des Maladies Neuro-musculaires (A.E.-L.), and Institut de Physiologie EA 3072, Service de Physiologie et d'Explorations Fonctionnelles (B.G., A.M.), Hôpitaux Universitaires de Strasbourg; Département de Médecine Interne et Immunologie Clinique (Y.A., O.B., B.H., K.M.), Centre de Référence des Maladies Neuro-Musculaires Paris Est, Assistance Publique-Hôpitaux de Paris (AP-HP), DHU I2B, Sorbonne Universités UPMC Univ Paris 06, Inserm, UMR 974, Centre de Recherche en Myologie, Hôpital Universitaire Pitié-Salpêtrière; Département de Neuropathologie (S.L.-L.), Centre de Référence des Maladies Neuro-Musculaires Paris Est, Hôpital Universitaire Pitié-Salpêtrière; Fédération de Médecine Translationnelle de Strasbourg (B.L., J.-E.G., B.G., J.S., A.M.), Université de Strasbourg; Département de Rhumatologie (D.W.), Hôpital Universitaire de Besançon, France; Département de Rhumatologie (C.V.F.), Hôpital Universitaire Sart-Tilman, Liège, Belgium; Departement de Rhumatologie (N.P.), Hôpital Universitaire de Bordeaux; Departement de Médicine Interne (E.M.), Hôpital Universitaire Louis Mourier, Colombes; Departement de Médicine Interne et Maladies Vasculaires (C.L.), Hôpital Universitaire d'Angers; Departement de Médicine Interne (O.H.), Hôpital Emile Muller, Mulhouse; Departement de Médicine Interne (J.M.), Hôpital Universitaire de Tours, France.

Objective: To refine the predictive significance of muscle granuloma in patients with myositis.

Methods: A group of 23 patients with myositis and granuloma on muscle biopsy (granuloma-myositis) from 8 French and Belgian centers was analyzed and compared with (1) a group of 23 patients with myositis without identified granuloma (control-myositis) randomly sampled in each center and (2) a group of 20 patients with sporadic inclusion body myositis (sIBM) without identified granuloma (control-sIBM).

Results: All but 2 patients with granuloma-myositis had extramuscular involvement, including signs common in sarcoidosis that were systematically absent in the control-myositis and the control-sIBM groups. Read More

Heart Fail Rev 2019 Dec 23. Epub 2019 Dec 23.

Department of Rheumatology, Medical University of Lodz, ul. Pieniny 30, 92-115, Łódź, Poland.

This review presents a detailed study of original researches and previously published reviews concerning cardiovascular involvement in idiopathic inflammatory myopathies (IIM). We aimed to summarize the current knowledge on the cardiac involvement in IIM, evaluate its impact on mortality and indicate areas still awaiting to be investigated. We searched MEDLINE database (until January 2019) and the reference lists of articles. Read More

Clin Neurophysiol 2020 Feb 6;131(2):368-371. Epub 2019 Dec 6.

Yale School of Medicine, Department of Neurology, 15 York Street, LCI 9, P.O. Box 208108, New Haven, CT 06519, USA. Electronic address:

Objective: To assess the value of electrical impedance myography (EIM) in inclusion body myositis (IBM).

Methods: Patients with clinically defined IBM and healthy controls (HC) of similar age group were recruited. Each participant underwent manual muscle testing (MMT), 6-min walk test (6MWT), handgrip dynamometry, and IBM-functional rating scale assessment (IBM-FRS). Read More

Clin Orthop Relat Res 2020 Mar;478(3):593-606

B. S. Baker, M. S. Stannard, D. L. Duren, J. L. Cook, J. P. Stannard, Department of Orthopaedic Surgery, University of Missouri, Columbia, MO, USA B. S. Baker, D. L. Duren, J. L. Cook, J. P. Stannard, Missouri Orthopedic Institute, Columbia, MO, USA M. S. Stannard, Department of Biomedical Sciences and Pathobiology, University of Missouri, Columbia, MO, USA D. L. Duren, Department of Pathology and Anatomical Sciences, University of Missouri, Columbia, MO, USA.

Background: Blood flow restriction (BFR) is a process of using inflatable cuffs to create vascular occlusion within a limb during exercise. The technique can stimulate muscle hypertrophy and improve physical function; however, most of these studies have enrolled healthy, young men with a focus on athletic performance. Furthermore, much of the information on BFR comes from studies with small samples sizes, limited follow-up time, and varied research designs resulting in greater design, selection, and sampling bias. Read More

Arthritis Res Ther 2019 12 18;21(1):293. Epub 2019 Dec 18.

Department of Sports Science and Clinical Biomechanics, SDU Muscle Research Cluster (SMRC), University of Southern Denmark, Odense, Denmark.

Background: Sporadic inclusion body myositis (sIBM) is clinically characterised by progressive proximal and distal muscle weakness and impaired physical function while skeletal muscle tissue displays abnormal cellular infiltration of T cells, macrophages, and dendritic cells. Only limited knowledge exists about the effects of low-load blood flow restriction exercise in sIBM patients, and its effect on the immunological responses at the myocellular level remains unknown. The present study is the first to investigate the longitudinal effects of low-load blood flow restriction exercise on innate and adaptive immune markers in skeletal muscle from sIBM patients. Read More

J Clin Neurosci 2020 Feb 12;72:244-251. Epub 2019 Dec 12.

Department of Neurology, Gunma University Graduate School of Medicine, Maebashi, Japan. Electronic address:

In clinical settings, it is often difficult to distinguish inclusion body myositis (IBM) from other neuromuscular diseases. In order to clarify clinically useful characteristics for making the differential diagnosis of IBM, we performed clinical, epidemiological, and neuroimaging analyses in patients with various types of neuromuscular disorders. We enrolled 333 patients with myopathy and 12 patients with amyotrophic lateral sclerosis (ALS) who had been hospitalized in our department from January 1, 1979, to December 31, 2018. Read More

Int J Gen Med 2019 5;12:465-474. Epub 2019 Dec 5.

School of Medicine, The University of Notre Dame, Fremantle, Western Australia, Australia.

Dysphagia in inclusion body myositis (IBM) is common and associated with increased mortality and morbidity due to aspiration pneumonia, malnutrition and dehydration. There is currently no consensus on treatment of dysphagia in IBM and outcomes are variable depending on timing of intervention, patient preference and available expertise. There is a paucity of research exploring the pathophysiology of dysphagia in IBM and appropriate investigations. Read More

Semin Arthritis Rheum 2020 06 16;50(3):498-502. Epub 2019 Nov 16.

Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH 44195, USA.

Objectives: To describe two cases of anti-PM-Scl antibody-associated systemic sclerosis with evidence of inclusion body myositis on muscle biopsy.

Methods: Two female patients with anti-PM-Scl antibody-associated systemic sclerosis developed progressive proximal myopathy. Both patients had profound muscle weakness that was refractory to treatment with glucocorticoids with or without other oral immunosuppressive agents. Read More

Continuum (Minneap Minn) 2019 Dec;25(6):1586-1598

Purpose Of Review: This article reviews the clinical, laboratory, and histopathologic features of sporadic inclusion body myositis (IBM) and explores its pathogenic overlap with inherited myopathies that have IBM-like pathology.

Recent Findings: Sporadic IBM is the most common acquired muscle disease in patients older than 50 years of age and is becoming more prevalent because of the increasing age of the population, the emerging development of more inclusive diagnostic criteria, and the advent of a diagnostic autoantibody. No effective therapy is known, and the pathogenic mechanism remains unclear. Read More

Eur J Neurol 2020 Apr 15;27(4):709-715. Epub 2019 Dec 15.

Neuromuscular and Rare Disease Unit, Department of Neuroscience, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.

Background And Purpose: The aim was to assess the value of insoluble PABPN1 muscle fibre nuclei accumulation in the diagnosis of atypical cases of oculopharyngeal muscular dystrophy (OPMD).

Methods: Muscle biopsies from a selected cohort of 423 adult patients from several Italian neuromuscular centres were analysed by immunofluorescence: 30 muscle biopsies of genetically proven OPMD, 30 biopsies from patients not affected by neuromuscular disorders, 220 from genetically undiagnosed patients presenting ptosis or swallowing disturbances, progressive lower proximal weakness and/or isolated rimmed vacuoles at muscle biopsy and 143 muscle biopsies of patients affected by other neuromuscular diseases.

Results: The detection of insoluble nuclear PABPN1 accumulation is rapid, sensitive (100%) and specific (96%). Read More