1,114 results match your criteria necrotizing myopathy


The Efficacy of Tocilizumab in the Treatment of Patients with Refractory Immune-Mediated Necrotizing Myopathies: An Open-Label Pilot Study.

Front Pharmacol 2021 16;12:635654. Epub 2021 Mar 16.

Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.

To evaluate the efficacy of tocilizumab (TCZ) in adult patients with refractory immune-mediated necrotizing myopathies (IMNMs) and investigate possible predictive biomarkers of the response to treatment with TCZ. Patients with refractory IMNM were enrolled in this open-label pilot observational study and received intravenous TCZ treatment. The clinical response was assessed after 6 months of TCZ treatment according to the 2016 American College of Rheumatology-European League Against Rheumatism (ACR-EULAR) response criteria for adult dermatomyositis and polymyositis. Read More

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Rituximab in the treatment of immune-mediated necrotizing myopathy: a review of case reports and case series.

Ther Adv Neurol Disord 2021 12;14:1756286421998918. Epub 2021 Mar 12.

Department of Rheumatology and Immunology, Nanchong Central Hospital, Nanchong, Sichuan, China.

Immune-mediated necrotizing myopathy (IMNM) is a group of immune-related myopathies characterized by progressive proximal muscle weakness, extremely high serum creatine kinase (CK) levels, and necrotic muscle fibers with a relative lack of inflammation. Treatment of IMNM is challenging, with most cases refractory to high-dose steroids in combination with multiple immunotherapies. The role of rituximab (RTX) for IMNM has been explored in isolated case reports and small series. Read More

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[A case of anti-Th/To antibody-positive systemic sclerosis with muscle symptoms and interstitial pneumonia].

Rinsho Shinkeigaku 2021 Mar 25. Epub 2021 Mar 25.

Internal Medicine, Cardiovascular, Respiratory and Neurology Division, Asahikawa Medical University.

A 62-year-old Japanese man with swollen fingers and walking difficulty due to myalgia and muscle weakness in proximal limb muscles was admitted to our hospital. Serum creatine kinase was remarkably increased (7,380 U/l) and rapidly progressing interstitial pneumonia developed. Muscle biopsy showed necrotic and regenerating fibers without mononuclear infiltration and fibrosis. Read More

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Asymptomatic necrotizing myositis in a young male with progressive interstitial lung disease.

Respir Med Case Rep 2021 2;32:101374. Epub 2021 Mar 2.

Division of Pulmonary and Critical Care, University of Rochester Medical Center, USA.

Necrotizing autoimmune myopathy (NAM) is a rare inflammatory process characterized by bilateral proximal muscle weakness and elevated creatinine kinase levels. It is one of the idiopathic inflammatory myopathies. It can be associated with anti-signal recognition particle (SRP) antibody which is commonly seen in middle-aged females. Read More

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Cardiac arrest in anti-mitochondrial antibody associated inflammatory myopathy.

Oxf Med Case Reports 2021 Mar 8;2021(3):omaa150. Epub 2021 Mar 8.

Copenhagen Lupus and Vasculitis Clinic, Centre for Rheumatology and Spine Diseases, Rigshospitalet, University of Copenhagen, Denmark.

Insight into predictors of cardiac involvement in inflammatory myopathies is sparse. A negative prognostic role of anti-mitochondrial antibodies (AMA) has been noticed and is supported by the current case. We describe a male patient who at the age 40 suffered a cardiac arrest and over the following months experienced progressive heart failure, arrhythmias and proximal muscle weakness. Read More

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Challenges in Treating Statin-Associated Necrotizing Myopathy.

Case Rep Rheumatol 2021 24;2021:8810754. Epub 2021 Feb 24.

Department of Rheumatology, Allegheny Health Network, Pittsburgh, USA.

Myalgia and mild elevation in muscle enzymes are common side effects of statin therapy. While these symptoms are generally self-limited, in rare cases, statin use is associated with an immune-mediated necrotizing myopathy caused by development of autoantibodies against HMG-CoA reductase. The primary presenting symptom of this condition is progressive symmetric proximal weakness that does not abate or worsens even after cessation of statin therapy and is associated with markedly elevated creatine kinase (CK) levels. Read More

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February 2021

Neurological Consequences of SARS-CoV-2 Infection and Concurrence of Treatment-Induced Neuropsychiatric Adverse Events in COVID-19 Patients: Navigating the Uncharted.

Front Mol Biosci 2021 18;8:627723. Epub 2021 Feb 18.

Drug Discovery and Development Centre (H3D), University of Cape Town, Rondebosch, South Africa.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) binds to the angiotensin-converting enzyme 2 (ACE2) receptor and invade the human cells to cause COVID-19-related pneumonia. Despite an emphasis on respiratory complications, the evidence of neurological manifestations of SARS-CoV-2 infection is rapidly growing, which is substantially contributing to morbidity and mortality. The neurological disorders associated with COVID-19 may have several pathophysiological underpinnings, which are yet to be explored. Read More

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February 2021

Atorvastatin-Induced Necrotizing Myopathy and its Response to Combination Therapy.

Cureus 2021 Jan 28;13(1):e12957. Epub 2021 Jan 28.

Internal Medicine, University of Nevada Reno School of Medicine, Reno, USA.

Atorvastatin is the most commonly used statin medication to decrease cholesterol levels and prevent atherosclerosis. Myopathy is a reported side effect of atorvastatin which can happen even after more than six months after starting the medication. The side effect on the muscle tissue can range from simple reversible myalgia to respiratory muscle compromise. Read More

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January 2021

Marked Respiratory Failure in an Ambulant Patient with Immune-mediated Necrotizing Myopathy and Anti-Kv1.4 and Anti-titin Antibodies.

Intern Med 2021 Mar 1. Epub 2021 Mar 1.

Department of Neurology, Graduate School of Medicine, Chiba University, Japan.

We herein report a case of seronegative immune-mediated necrotizing myopathy (IMNM) concurrent with anti-Kv1.4 and anti-titin antibodies. A 72-year-old Japanese woman presented with a 29-year history of fluctuating high serum creatine kinase (CK) levels followed by intermittent ptosis and respiratory muscle weakness. Read More

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The Novel Coronavirus Infection (COVID-19) and Nervous System Involvement: Mechanisms of Neurological Disorders, Clinical Manifestations, and the Organization of Neurological Care.

Neurosci Behav Physiol 2021 Feb 18:1-8. Epub 2021 Feb 18.

Pirogov Russian National Research Medical University, Russian Ministry of Health, Moscow, Russia.

The new coronavirus SARS-CoV-2 and the disease it causes COVID-19 involves not only respiratory system damage, but can also lead to disorders of the central and peripheral nervous system, as well as the muscular system. This article presents published data and our own observations on the course of neurological disorders in COVID-19 patients. There is a relationship between the severity of COVID-19 and the severity and frequency of neurological manifestations. Read More

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February 2021

Immunotherapy reversed myopathy but not cardiomyopathy in a necrotizing autoimmune myopathy patient with positive anti-SRP and MDA-5 autoantibodies.

BMC Cardiovasc Disord 2021 Feb 12;21(1):88. Epub 2021 Feb 12.

Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1095, Jiefang Avenue, Qiaokou District, Wuhan, China.

Background: Necrotizing autoimmune myopathy (NAM) is pathologically characterized by myofiber necrosis and regeneration with paucity or absence of inflammatory cells in muscle biopsy. Two autoantibodies, namely anti-signal recognition particle (SRP)-antibodies and anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR)-antibodies, are typically specific with NAM. Anti-SRP-positive NAM can be associated with cardiomyopathy which responds well to immunotherapy. Read More

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February 2021

Genetic Modifiers of Hereditary Neuromuscular Disorders and Cardiomyopathy.

Cells 2021 Feb 8;10(2). Epub 2021 Feb 8.

Heart, Lung and Vascular Institute, Division of Cardiovascular Health and Disease, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, OH 45267, USA.

Novel genetic variants exist in patients with hereditary neuromuscular disorders (NMD), including muscular dystrophy. These patients also develop cardiac manifestations. However, the association between these gene variants and cardiac abnormalities is understudied. Read More

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February 2021

[A Case of Immune-Mediated Necrotizing Myopathy Associated with Primary Sjögren Syndrome].

Brain Nerve 2021 Feb;73(2):183-187

Department of Neurology, Fukuoka University Faculty of Medicine.

A 66-year-old woman visited our hospital complaining of shortness of breath during exertion and progressive weakness in all her limb muscles. On admission, we noted muscle weakness in her trunk and in her proximal limb muscles, although, her muscle MRI showed no remarkable findings. However, her serum CK level (2,747U/L) was above the normal range. Read More

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February 2021

[Clinicopathological Features of Myositis and Necrotizing Myopathy: How to Distinguish between Myositis and Muscular Dystrophy on Muscle Pathology].

Brain Nerve 2021 Feb;73(2):147-159

Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry.

In the field of neurology, idiopathic inflammatory myopathy has been classified into four sub-categories, namely, dermatomyositis, anti-synthetase syndrome, inclusion body myositis, and immune-mediated necrotizing myopathy (IMNM), based upon histological and serological findings. Among them, IMNM has features similar to muscular dystrophy, and it may thus be difficult to differentiate between these two conditions, not only clinically but also pathologically, especially in chronic cases and pediatric patients. This is partly because the main pathological feature of both IMNM and muscular dystrophy is myofiber necrosis and regeneration. Read More

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February 2021

[Immune-Mediated Necrotizing Myopathy: IMNM].

Brain Nerve 2021 Feb;73(2):127-136

Department of Neurology, JA Toride Medical Center.

Immune-mediated necrotizing myopathy (IMNM) is a heterogeneous disease that presents with subacute progressive proximal dominant muscle weakness clinically and prominent necrotic muscle fibers without invasion of inflammatory cells pathologically. IMNM is a disease that exclusively affects skeletal muscles, so the serum creatine kinase level usually increases prominently. It is thought to be an autoimmune disease because of the presence of two autoantibodies, anti-SRP and anti-HMGCR. Read More

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February 2021

Clinical utility of anti-cytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies.

Ann Clin Transl Neurol 2021 03 8;8(3):571-578. Epub 2021 Feb 8.

Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.

Objective: To define the clinicopathologic features and diagnostic utility associated with anti-cytosolic 5'-nucleotidase 1A (NT5C1A) antibody seropositivity in idiopathic inflammatory myopathies (IIMs).

Methods: Anti-NT5C1A antibody status was clinically tested between 2014 and 2019 in the Washington University neuromuscular clinical laboratory. Using clinicopathologic information available for 593 patients, we classified them as inclusion body myositis (IBM), dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy (IMNM), nonspecific myositis, or noninflammatory muscle diseases. Read More

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The Biosafety and Risk Management in Preparation and Processing of Cerebrospinal Fluid and Other Neurological Specimens With Potential Coronavirus Infection.

Front Neurol 2020 20;11:613552. Epub 2021 Jan 20.

Department of Internal Medicine, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chiayi, Taiwan.

The recent outbreak of coronavirus disease 2019 (COVID-19), caused by Severe Acute Respiratory Syndrome Coronavirus 2, has become a global threat. Due to neurological manifestations presented throughout the coronavirus disease process, the potential involvement of COVID-19 in central nervous system has attracted considerable attention. Notably, the neurologic system could be widely affected, with various complications such as acute cerebrovascular events, encephalitis, Guillain-Barré syndrome, and acute necrotizing hemorrhagic encephalopathy. Read More

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January 2021

Treatment of Statin-Induced Necrotizing Autoimmune Myopathy With Glucocorticoid Monotherapy.

Cureus 2020 Dec 14;12(12):e12086. Epub 2020 Dec 14.

Rheumatology, Waterbury Hospital, Waterbury, USA.

Statins are widely prescribed medications to prevent cardiovascular events such as myocardial infarction and stroke. Both myalgia and myopathy are well-known potential side effects of statins. However, a rare and severe form of statin-induced necrotizing autoimmune myopathy (SINAM) has recently been described and can lead to debilitating weakness, often requiring immunosuppressive therapy. Read More

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December 2020

Dermatomyositis-Induced Rhabdomyolysis With Features of Necrotizing Myopathy and Acute Inflammatory Demyelinating Polyneuropathy in an Epstein-Barr Virus Infected Patient.

Cureus 2020 Dec 14;12(12):e12077. Epub 2020 Dec 14.

Internal Medicine, Rowan University School of Osteopathic Medicine, Stratford, USA.

Dermatomyositis (DM) is an autoimmune inflammatory myopathy characterized by features of a typical rash, proximal muscle weakness, and evidence of muscle inflammation. Acute inflammatory demyelinating polyneuropathy (AIDP) is an autoimmune peripheral nerve disease characterized by myelin damage and progressive areflexic weakness and sensory changes. AIDP can be precipitated by viral infections such as Epstein-Barr virus (EBV). Read More

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December 2020

Maintenance treatment with subcutaneous immunoglobulins in the long-term management of anti-HMCGR myopathy.

Neuromuscul Disord 2021 02 28;31(2):134-138. Epub 2020 Dec 28.

Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal and Infantile Sciences (DINOGMI), University of Genova, Largo P. Daneo3, 16132 Genova, Italy; IRCCS Ospedale Policlinico San Martino, Genova, Italy. Electronic address:

We describe the clinical response to long-term subcutaneous immunoglobulins (SCIg) in anti-3‑hydroxy-3-methyl-glutaryl-coenzyme-A-reductase (anti-HMCGR) myopathy previously treated with intravenous immunoglobulins (IVIg). We collected data from patients affected by anti-HMGCR myopathy, switched from IVIg to SCIg therapy, after achieving clinical stabilization. The Medical Research Council sum score, creatine kinase (CK) levels, and anti-HMGCR antibodies were used to assess the response. Read More

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February 2021

Inflammatory myopathies: update on diagnosis, pathogenesis and therapies, and COVID-19-related implications.

Acta Myol 2020 Dec 1;39(4):289-301. Epub 2020 Dec 1.

Department of Neurology, Thomas Jefferson University, Philadelphia, PA USA and the Neuroimmunology Unit, National and Kapodistrian University University of Athens Medical School, Athens, Greece.

The inflammatory myopathies constitute a heterogeneous group of acquired myopathies that have in common the presence of endomysial inflammation. Based on steadily evolved clinical, histological and immunopathological features and some autoantibody associations, these disorders can now be classified in five characteristic subsets: Dermatomyositis (DM) Polymyositis (PM), Necrotizing Autoimmune Myositis (NAM), Anti-synthetase syndrome-overlap myositis (Anti-SS-OM), and Inclusion-Body-Myositis (IBM). Each inflammatory myopathy subset has distinct immunopathogenesis, prognosis and response to immunotherapies, necessitating the need to correctly identify each subtype from the outset to avoid disease mimics and proceed to early therapy initiation. Read More

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December 2020

Statin-induced necrotizing autoimmune myopathy.

Proc (Bayl Univ Med Cent) 2020 Oct 21;34(1):185-186. Epub 2020 Oct 21.

Department of Internal Medicine, State University of New York Upstate Medical University, Syracuse, New York.

Statins are the most widely used class of drug in the United States. They lower blood cholesterol levels by inhibiting 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase. Common side effects include myalgias and a mild increase in liver function tests. Read More

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October 2020

Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report.

SAGE Open Med Case Rep 2020 29;8:2050313X20984120. Epub 2020 Dec 29.

Division of Rheumatology, Department of Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. Read More

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December 2020

Neurological manifestations associated with SARS-CoV-2 and other coronaviruses: A narrative review for clinicians.

Rev Neurol (Paris) 2021 Jan-Feb;177(1-2):51-64. Epub 2020 Dec 16.

Department of Neurology, centre hospitalier de Saint-Denis, hôpital Delafontaine, 93200 Saint-Denis, France. Electronic address:

Introduction: The past two decades have been marked by three epidemics linked to emerging coronaviruses. The COVID-19 pandemic highlighted the existence of neurological manifestations associated with SARS-CoV-2 infection and raised the question of the neuropathogenicity of coronaviruses. The aim of this review was to summarize the current data about neurological manifestations and diseases linked to human coronaviruses. Read More

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February 2021

Pregabalin-Induced Myopathy in a Double Lung Transplant Recipient.

Cureus 2020 Dec 6;12(12):e11935. Epub 2020 Dec 6.

Internal Medicine, University of Kentucky College of Medicine, Lexington, USA.

Pregabalin is a gamma-aminobutyric acid (GABA) derivative that was commercially approved by the Food and Drug Administration (FDA) in 2004. It is commonly used in the treatment of diabetic neuropathy, peripheral neuropathy, and spinal cord injury. We present the case of a 36-year-old Caucasian male double lung transplant recipient who presented with an 18-month history of fatigue and muscle weakness. Read More

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December 2020

A Clinical Image of Autoantibody-Negative Immune-Mediated Necrotizing Myopathy With Concurrent Thymoma-Associated Myasthenia Gravis.

J Clin Rheumatol 2020 Dec 24;Publish Ahead of Print. Epub 2020 Dec 24.

From the Department of Internal Medicine, University of California, San Francisco-Fresno, Fresno, CA.

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December 2020

Statin-Associated Necrotizing Myopathy: A Feared Complication.

Cureus 2020 Nov 24;12(11):e11689. Epub 2020 Nov 24.

Internal Medicine, Hartford Hospital, Hartford, USA.

Statins are a group of frequently-prescribed drugs with proven cardiovascular risk-benefit. The most common adverse effects include weakness and myalgias. However, prescribers need to be aware of a less common complication, statin-associated necrotizing myopathy, which can occur at any time during the treatment course and has been found to be <0. Read More

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November 2020

A case of statin-associated immune-mediated necrotizing myopathy with atypical biopsy features.

Eur J Rheumatol 2021 Jan 1;8(1):36-39. Epub 2021 Jan 1.

Department of Medicine, Harvard Medical School, Boston, MA, USA.

Statin-associated immune-mediated necrotizing myopathy (IMNM) is a rare presentation of a statin-associated myopathy. Patients usually present with muscle weakness and pain in the setting of statin use with elevated creatine kinase (CK) levels and a positive anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody. Muscle biopsies typically show necrosis, CD68+ macrophages, and minimal lymphocytes. Read More

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January 2021

Polymyositis: is there anything left? A retrospective diagnostic review from a tertiary myositis centre.

Rheumatology (Oxford) 2020 Dec 24. Epub 2020 Dec 24.

National Institute for Health Research Manchester Musculoskeletal Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, University of Manchester, Manchester, UK.

Objective: The current classification criteria for idiopathic inflammatory myopathy (IIM) retain PM as a major disease subgroup. However, evolution in the understanding of IIM has suggested that many of these patients could be better described as having an alternative diagnosis. In the present study, we apply the latest understanding of IIM subtyping to retrospectively review PM diagnoses in a large cohort of IIM patients. Read More

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December 2020

Diagnostic modelling and therapeutic monitoring of immune-mediated necrotizing myopathy: role of electrical myotonia.

Brain Commun 2020 13;2(2):fcaa191. Epub 2020 Dec 13.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Delayed diagnosis of immunemediated necrotizing myopathy leads to increased morbidity. Patients with the chronic course without 3-hydroxy-3-methylglutaryl-coenzyme-A reductase-IgG or signal recognition particle-IgG are often challenging to diagnose. Immunotherapy response can also be difficult to assess. Read More

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December 2020