365 results match your criteria immune-mediated necrotizing


Aberrantly Expressed Galectin-9 Is Involved in the Immunopathogenesis of Anti-MDA5-Positive Dermatomyositis-Associated Interstitial Lung Disease.

Front Cell Dev Biol 2021 25;9:628128. Epub 2021 Mar 25.

Department of Rheumatology, Beijing Key Lab for Immune-Mediated Inflammatory Diseases, China-Japan Friendship Hospital, Beijing, China.

Background: Dermatomyositis (DM) associated rapidly progressive interstitial lung disease (RP-ILD) has high mortality rate and poor prognosis. Galectin-9 (Gal-9) plays multiple functions in immune regulation. We investigated Gal-9 expression in DM patients and its association with DM-ILD. Read More

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An Autopsy Case of Antibody-negative Immune-mediated Necrotizing Myopathy with Severe Cardiac Involvement.

Intern Med 2021 Apr 12. Epub 2021 Apr 12.

Division of Cardiology, Internal Medicine III, Hamamatsu University School of Medicine, Japan.

A 41-year-old man was admitted with a chief complaint of dyspnea. Echocardiography showed diffuse severe hypokinesis in the left ventricle. Although his heart failure improved, high creatine kinase levels persisted. Read More

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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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A Severe Necrotizing Inflammatory Reaction of Leg Wounds Following Autologous Peripheral Blood Total Nucleated Cells Treatment in an Old Patient With Rheumatoid Arthritis and No-Option Chronic Limb-Threatening Ischemia: Is Cell Therapy Suitable for All Patients?

Int J Low Extrem Wounds 2021 Mar 15:1534734621997570. Epub 2021 Mar 15.

Felice Lotti Hospital, Pontedera, Pisa, Toscana, Italy.

Chronic limb-threatening ischemia (CLTI) represents an unfavorable evolution of peripheral artery disease, characterized by pain at rest, ulceration, and gangrene and also by an increased risk of cardiovascular events, amputations, and death. According to scientific literature, in almost one third of cases affected by CLTI, defined as no-option CLTI patients, revascularization strategies are not feasible. In the past decade, several studies investigated the role of therapeutic angiogenesis through cell autologous therapy, administered through intramuscular injections or multiple local intralesional and perilesional injections. 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

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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[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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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

Recurrent necrotizing cellulitis, multi-organ autoimmune disease and humoral immunodeficiency due to a novel NFKB1 frameshift mutation.

Eur J Med Genet 2021 Mar 22;64(3):104144. Epub 2021 Jan 22.

Department of Dermatology, University Medical Center Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Germany. Electronic address:

Background: Mutations in NFKB1(nuclear factor of kappa light polypeptide gene enhancer in B-cells 1) are associated with a variety of clinical symptoms, including lymphadenopathy, splenomegaly, hepatomegaly, autoimmune haemolytic anaemia, arthralgia, recurrent respiratory tract infections and post-operative necrotizing cellulitis.

Case Presentation: We describe a case of a 47-year-old man, who presented with deep necrotizing cellulitis after incision of a submucous abscess by a dentist. Surgical intervention led to a massive progress. Read More

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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

Central and peripheral nervous system complications of COVID-19: a prospective tertiary center cohort with 3-month follow-up.

J Neurol 2021 Jan 13. Epub 2021 Jan 13.

Departments of Neurology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

Objective: To systematically describe central (CNS) and peripheral (PNS) nervous system complications in hospitalized COVID-19 patients.

Methods: We conducted a prospective, consecutive, observational study of adult patients from a tertiary referral center with confirmed COVID-19. All patients were screened daily for neurological and neuropsychiatric symptoms during admission and discharge. Read More

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January 2021

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

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

Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome.

Eur Heart J 2020 Dec 23. Epub 2020 Dec 23.

Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences, Sapienza University, Viale del Policlinico 155, 00161 Rome, Italy.

Aims : Necrotizing coronary vasculitis (NCV) is a rare entity usually associated to myocarditis which incidence, cause, and response to therapy is unreported.

Methods And Results : Among 1916 patients with biopsy-proven myocarditis, 30 had NCV. Endomyocardial samples were retrospectively investigated with immunohistochemistry for toll-like receptor 4 (TLR4) and real-time polymerase chain reaction (PCR) for viral genomes. Read More

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December 2020

Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure.

Front Neurol 2020 25;11:571716. Epub 2020 Nov 25.

Department of Neurology, University of Minnesota, Minneapolis, MN, United States.

Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from proximal more than distal muscle weakness for 3 months prior to admission. She was found to have acute systolic heart failure. Read More

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November 2020

Neovascular complications from cytomegalovirus necrotizing retinopathy in patients after haploidentical hematopoietic stem cell transplantation.

Retina 2020 Dec 10. Epub 2020 Dec 10.

Department of Ophthalmology & Clinical Center of Optometry, Peking University People's Hospital, Beijing 100044, China, Eye Diseases and Optometry Institute, Peking University People's Hospital, Beijing 100044, China, Beijing Key Laboratory of Diagnosis and Therapy of Retinal and Choroid Diseases, Beijing 100044, China, and College of Optometry, Peking University Health Science Center, Beijing 100191, China.

Purpose: To report the incidence and clinical features of neovascular complications from cytomegalovirus (CMV) necrotizing retinopathy in patients after haploidental hematopoietic stem cell transplantation (HHSCT).

Methods: Thirty-nine patients (58 eyes) of CMV necrotizing retinopathy after HHSCT in our institute between 2018 January and 2020 June were retrospectively reviewed and cases that developed neovascular complications during follow up were identified and described.

Results: Two (2 eyes) cases that developed neovascular glaucoma (NVG) from CMV necrotizing retinopathy were indentified. Read More

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December 2020

Immune checkpoint inhibitor-associated myopathy: a clinicoseropathologically distinct myopathy.

Brain Commun 2020 2;2(2):fcaa181. Epub 2020 Nov 2.

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

Immune checkpoint inhibitors have revolutionized the landscape of cancer treatment. Alongside their many advantages, they elicit immune-related adverse events, including myopathy, which potentially result in substantial morbidity if not recognized and treated promptly. Current knowledge of immune checkpoint inhibitor-associated myopathy is limited. Read More

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November 2020

Successful repair of combined tractional and rhegmatogenous retinal detachment with pars plana vitrectomy without scleral buckle in severe Behçet's disease.

J Surg Case Rep 2020 Nov 30;2020(11):rjaa468. Epub 2020 Nov 30.

Department of Ophthalmology, Loma Linda University, Loma Linda, CA, USA.

Behçet's disease (BD) is a multisystemic, immune-mediated occlusive vasculitis of unknown etiology with a chronic, relapsing remitting course. Ocular involvement is characterized by recurrent nongranulomatous uveitis with necrotizing obliterative vasculitis affecting both the anterior and posterior segments of the eye and often leads to blindness. We describe successful surgical management of a rare case of combined rhegmatogenous retinal detachment (RRD) and tractional retinal detachment (TRD) in a patient with Behçet's disease. Read More

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November 2020

Severe muscle damage with myofiber necrosis and macrophage infiltrates characterize anti-Mi2 positive dermatomyositis.

Rheumatology (Oxford) 2020 Nov 29. Epub 2020 Nov 29.

Unit of Rheumatology, Department of Emergency and Organ Transplantation, University of Bari.

Objective: The aim of our study was to investigate clinical and histopathological findings in adult DM patients positive for anti-Mi2 (anti-Mi2+) antibodies compared with DM patients negative for anti-Mi2 (anti-Mi2-).

Methods: Clinical data of adult DM patients, who fulfilled EULAR/ACR 2017 classification criteria, were gathered from electronic medical records of three tertiary Rheumatology Units. Histopathological study was carried out on 12 anti-Mi2+ and 14 anti-Mi2- muscle biopsies performed for diagnostic purpose. Read More

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November 2020

[Myositis: From classification to diagnosis].

Rev Med Interne 2020 Nov 25. Epub 2020 Nov 25.

Département de Médecine interne et immunologie clinique, Centre national de référence des maladies neuromusculaires, hôpital Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.

Idiopathic inflammatory myopathies, or IIM, are a group of acquired diseases that affect the muscle to a certain extent, and may also affect other organs. They include dermatomyositis, which can affect the muscle eventualy, with a typical skin rash; inclusion body myositis, with a purely muscular expression resulting in a slow progressive deficit; and the former group of "polymyositis", a misnomer that actually includes other categories of IIM, such as immune-mediated necrotizing myopathies, with a severe muscle involvement often presents from the onset of the disease; antisynthetase syndrome, which combines muscle damage, joint involvement and a potentially life-threatening lung disease; and overlapping myositis, which combines muscle damage with other organs involvement connected to another autoimmune disease. The diagnosis of IIM is based on rigorous clinical examination and interrogation, electromyographic data and immunological testing for myositis specific antibodies. Read More

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November 2020

Utilization of myositis antibody-specific panel for diagnosis, treatment, and evaluation of disease progression.

J Community Hosp Intern Med Perspect 2020 Sep 3;10(5):462-465. Epub 2020 Sep 3.

Greater Baltimore Medical Center, Internal Medicine Residency, Towson, MD, USA.

The idiopathic inflammatory myopathies (IIM) are rare sporadic disorders with an overall annual incidence of approximately 1 in 100,000 and with a higher incidence in women. IIM is an autoimmune process leading to muscle inflammation due to a 'dysfunctional adaptive immune response evidenced by cell-mediated myocytoxicity, a high prevalence of autoantibodies and overexpression of Major Histocompatibility (MHC) I and II molecules on the muscle sarcolemma'. These autoimmune processes can be appreciated as inflammatory infiltrates in muscle biopsies. Read More

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September 2020

What is New in Idiopathic Inflammatory Myopathies: Mechanisms and Therapies.

Ann Indian Acad Neurol 2020 Jul-Aug;23(4):458-467. Epub 2020 Apr 13.

Department of Neurology, Dr. L H Hiranandani Hospital, Mumbai, Maharashtra, India.

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders that cause muscle weakness and also have extramuscular manifestations involving various organ systems; namely the lung, skin, heart, and joints. Previously classified broadly as dermatomyositis (DM) and polymyositis now the spectrum of the disease has evolved into more clinical subtypes. There are now five clinicoserological subtypes recognized worldwide DM, antisynthetase syndrome (AS), overlap myositis (OM), immune mediated necrotizing myopathy (IMNM), and inclusion body myositis. Read More

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