scholarly journals Autoimmune necrotizing myositis. Presentation of a clinical case

2021 ◽  
pp. 24-28
Author(s):  
Sabrina Plou ◽  
María Elena Gaona ◽  
Víctor Caputo
Keyword(s):  
Muscle Weakness ◽  
Inflammatory Cell ◽  
Inflammatory Cell Infiltrate ◽  
Inflammatory Myopathies ◽  
Immune Mediated ◽  
Necrotizing Myositis ◽  
Upper And Lower Extremities ◽  
Back Ground ◽  
Systemic Disorder ◽  
Autoimmune Myopathy

Inflammatory myopathies (IM) or myositis are a heterogeneous group of muscle diseases of rare occurrence. Such diseases are characterized by inflammation of the different components of muscle tissue, which can occur either in isolation or, more commonly, as part of a systemic disorder. Immune-mediated necrotizing myopathies (IMNM) are a type of autoimmune myopathy characterized by proximal muscle weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy and infrequent extramuscular involvement. Even though there are clinical and histopathological similarities. The spectrum of inflammatory myopathies is considerably variable. Therefore, the performance of complementary studies is essential for the proper identification of the IM subtype to contribute accurately on treatment so determine the better prognosis). The present article shows the case of a young 29 years old, with no personal and family history background of autoimmune disease and no relevant pathological back-ground. The patient consulted the medical ward of the Institution with pain, functional impairment of upper and lower extremities, muscle weakness mainly located in the pectoral girdle area and, although to a lesser degree, in the pelvic girdle area. It was also associated with asthenia, tendency to drowsiness and hyporeactivity.

Vaccination as a possible trigger for immune-mediated necrotising myopathy

2021 ◽  
Vol 14 (5) ◽  
pp. e242095
Author(s):  
Julia Francesca Bonato Cavalcanti ◽  
Maria Beatriz Almeida Silva ◽  
Alzira Alves de Siqueira Carvalho
Keyword(s):  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Inflammatory Cell ◽  
Reference Value ◽  
Lower Limbs ◽  
Cervical Region ◽  
Allergic Reactions ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Autoimmune Myopathy

Immune-mediated necrotising myopathy is a rare autoimmune myopathy characterised by severe progressive muscle weakness, elevated levels of creatine kinase (CK), and necrosis with minimal inflammatory cell infiltration on muscle biopsy. We report a case of a previously healthy 42-year-old woman who presented with progressive muscle weakness 2 weeks after immunisation for yellow fever, tetanus/diphtheria and hepatitis B. Her symptoms started from the lower limbs and progressed to the upper limbs and cervical region associated with dysphagia, making her wheelchair bound. Electromyography showed a myopathic pattern, with a CK level of 12.177 U/L (reference value: 26–190 U/L), and biceps brachial muscle biopsy confirmed necrosis and regeneration fibres. The immunoblot test was positive for antisignal recognition particle. She was successfully treated with prednisone (1 mg/kg/day). Although considered safe, vaccines may cause allergic reactions or trigger autoimmune disorders. Currently, a causal relationship between them cannot be established.

Biological therapy in idiopathic inflammatory myopathies

2014 ◽  
Vol 155 (1) ◽  
pp. 3-10
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Csilla András ◽  
...  
Keyword(s):  
T Cells ◽  
Muscle Weakness ◽  
Biological Therapy ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Novel Therapeutic

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders. Orv. Hetil., 2014, 155(1), 3–10.

Polymyositis and dermatomyositis

2013 ◽  
Author(s):  
Hector Chinoy ◽  
Robert G. Cooper
Keyword(s):  
Muscle Weakness ◽  
Inflammatory Cell ◽  
Evidence Base ◽  
Inflammatory Myopathies ◽  
Connective Tissue Disorders ◽  
Antibody Testing ◽  
Muscle Enzymes ◽  
Skeletal Muscle Weakness ◽  
Form Part ◽  
Prompt Diagnosis

Polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM) form part of the idiopathic inflammatory myopathies (IIM), a heterogeneous group of rare autoimmune diseases characterized by an acquired proximal muscle weakness, raised muscle enzymes (including creatine kinase), inflammatory cell infiltrates in muscle biopsy tissue, electrophysiological abnormalities, and presence of circulating myositis-specific/myositis-associated autoantibodies. The underlying aetiology of IIM is poorly understood, but likely involves interactions between environmental and genetic risk factors. Myositis may also manifest in association with other connective tissue disorders. The predominant clinical presentation of IIM is skeletal muscle weakness, but many extramuscular features can also occur. Access to good neuropathological support is essential in securing an accurate IIM diagnosis and excluding non-inflammatory myopathies, although IBM is often difficult to distinguish from PM. Antibody testing can help define IIM clinical subtypes, including cancer-associated myositis, predict prognosis, and help in optimizing treatment decisions. MRI can be invaluable for differentiating disease activity from damage, and detecting treatment-induced interval changes. Therapeutic effectiveness of new and existing treatments (where the evidence base remains poor) depends on making a prompt diagnosis and initiating early and appropriately aggressive treatment to prevent establishment of muscle damage. This chapter attempts to summarize the salient features of IIM and update the reader about currently used diagnostics and treatment paradigms in this rare and understudied disease.

In the idiopathic inflammatory myopathies (IIM), do reactive oxygen species (ROS) contribute to muscle weakness?

2015 ◽  
Vol 74 (7) ◽  
pp. 1340-1346 ◽  
Author(s):  
Adam P Lightfoot ◽  
Anne McArdle ◽  
Malcolm J Jackson ◽  
Robert G Cooper
Keyword(s):  
Reactive Oxygen Species ◽  
Muscle Weakness ◽  
Inflammatory Cell ◽  
Immune Cell ◽  
Ros Generation ◽  
Muscle Dysfunction ◽  
Idiopathic Inflammatory Myopathies ◽  
Oxygen Species ◽  
Inflammatory Myopathies

The idiopathic inflammatory myopathies (IIMs) are a group of rare autoimmune disorders, collectively known as myositis. Affected patients present with proximal muscle weakness, which usually improves following treatment with immunosuppressants, but often incompletely so, thus many patients remain weak. IIMs are characterised histologically by inflammatory cell infiltrates into skeletal muscle and overexpression of major histocompatibility complex I on muscle cell surfaces. Although inflammatory cell infiltrates represent a major feature of myositis there is growing evidence that muscle weakness correlates only poorly with the degree of cellular infiltration, while weakness may in fact precede such infiltrations. The mechanisms underpinning such non-immune cell mediated weakness in IIM are poorly understood. Activation of the endoplasmic reticulum stress pathways appears to be a potential contributor. Data from non-muscle cells indicate that endoplasmic reticulum stress results in altered redox homeostasis capable of causing oxidative damage. In myopathological situations other than IIM, as seen in ageing and sepsis, evidence supports an important role for reactive oxygen species (ROS). Modified ROS generation is associated with mitochondrial dysfunction, depressed force generation and activation of muscle catabolic and autophagy pathways. Despite the growing evidence demonstrating a key role for ROS in skeletal muscle dysfunction in myopathologies other than IIM, no research has yet investigated the role of modified generation of ROS in inducing the weakness characteristic of IIM. This article reviews current knowledge regarding muscle weakness in the absence of immune cells in IIM, and provides a background to the potential role of modified ROS generation as a mechanism of muscle dysfunction. The authors suggest that ROS-mediated mechanisms are potentially involved in non-immune cell mediated weakness seen in IIM and outline how these mechanisms might be investigated in this context. This appears a timely strategy, given recent developments in targeted therapies which specifically modify ROS generation.

Rapidly Progressive Proximal Weakness

2021 ◽  
pp. 152-153
Author(s):  
Teerin Liewluck ◽  
Margherita Milone
Keyword(s):  
Creatine Kinase ◽  
Mycophenolate Mofetil ◽  
Intravenous Immunoglobulin ◽  
Muscle Weakness ◽  
Creatine Kinase Level ◽  
Coenzyme A ◽  
Immune Mediated ◽  
Necrotizing Autoimmune Myopathy ◽  
History Of ◽  
Autoimmune Myopathy

A 53-year-old woman had development of subacute-onset muscle weakness resulting in difficulty climbing stairs, rising from a chair, and reaching over her shoulders. She reported no dysphagia, dysarthria, dyspnea, or diplopia. She also disclosed no rash, joint pain, or urine discoloration. She had no history of statin exposure. There was no family history of neuromuscular disorders, early cataracts, cardiac arrhythmia, or cardiomyopathy. Two months of treatment with prednisone had resulted in no clinical improvement. Neurologic examination indicated moderate neck flexor, shoulder, and hip girdle muscle weakness, with sparing of cranial muscles. There was no action- or percussion-induced myotonia. Needle electromyography showed short-duration, low-amplitude, and complex motor unit potentials, predominantly affecting proximal muscles, associated with fibrillation potentials and myotonic discharges in proximal and axial muscles. Her creatine kinase level was increased. Biopsy of the left quadriceps showed variation in muscle fiber size, a moderate increase in internalized nuclei, fiber splitting, and scattered necrotic and regenerating fibers. There was a mild increase in perimysial fibrous and fatty connective tissue. 3-Hydroxy-3-methylglutaryl–coenzyme A reductase antibodies were strongly positive. The patient was diagnosed with hydroxy-3-methylglutaryl–coenzyme A reductase antibody–positive necrotizing autoimmune myopathy. The patient received intravenous immunoglobulin and mycophenolate mofetil while continuing prednisone. At 1-year follow-up, she had no weakness, and her creatine kinase value was normal while she continued taking prednisone, mycophenolate mofetil, and intravenous immunoglobulin. Necrotizing autoimmune myopathy, or immune-mediated necrotizing myopathy, is a subtype of immune-mediated myopathy, clinically characterized by subacute, progressive, proximal limb weakness and persistently increased creatine kinase level. Pathologically, it is characterized by myonecrosis with minimal or no inflammation. One-third of patients with necrotizing autoimmune myopathy have myalgia.

Necrotizing autoimmune myopathy

2012 ◽  
Vol 153 (38) ◽  
pp. 1502-1507 ◽  
Author(s):  
Levente Bodoki ◽  
Melinda Vincze ◽  
Tibor Hortobágyi ◽  
Zoltán Griger ◽  
Karolina Cseri ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Rare Entity ◽  
Separate Entity ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Systemic Autoimmune Diseases ◽  
Necrotizing Autoimmune Myopathy ◽  
Autoimmune Myopathy ◽  
Very High

Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. One of them is the subgroup of necrotizing autoimmune myopathy, which has recently been recognized as a separate entity. In addition to the typical symmetrical muscle weakness, it is characterized by very high creatine kinase levels, myopathic triad in the electromyography, and myocyte necrosis without significant inflammation. The paper aims to review this rare entity, which has to be diagnosed and treated quickly in every case. Orv. Hetil., 2012, 153, 1502–1507.

scholarly journals Juvenile Dermatomyositis and the Inflammatory Myopathies

2020 ◽  
Vol 40 (03) ◽  
pp. 342-348
Author(s):  
Collin Swafford ◽  
E. Steve Roach
Keyword(s):  
Muscle Weakness ◽  
Muscle Injury ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Rimmed Vacuoles ◽  
Immune Mediated ◽  
Primary Disorder ◽  
Tissue Changes

AbstractThe 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. Children with JDM present with proximal muscle weakness and characteristic rashes. The presentation is similar in children and adults, but JDM is a primary disorder and the adult form often is concerning for a paraneoplastic syndrome. Proximal muscle weakness occurs with dermatomyositis, polymyositis, and immune-mediated necrotizing myopathy, but the latter two conditions have no dermatologic findings or distinct tissue changes which set them apart from dermatomyositis. Inclusion body myositis, also included in the inflammatory myopathies, presents with more distal involvement, and microscopically exhibits identifiable rimmed vacuoles. We review key features of these disorders, focusing in more detail on JDM because it is more often encountered by the child neurologist.

A Case of Immune-Mediated Necrotizing Myopathy With Associated Skeletal Muscle Involvement by Sarcoid Granulomata: A Rare Association

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S69-S69
Author(s):  
Shalla Akbar ◽  
Sandhya Dasaraju ◽  
Osama Elkadi
Keyword(s):  
Skeletal Muscle ◽  
Muscle Weakness ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Skeletal Muscle Involvement

Abstract Skeletal muscle involvement by noncaseating granulomata occurs in a variety of conditions, including sarcoidosis, infections, and rarely in association with primary inflammatory myopathies such as inclusion body myositis (IBM) and dermatomyositis (DM). Sarcoid myopathy is typically asymptomatic; however, a picture of acute myositis with proximal muscle weakness has been described. Immune-mediated necrotizing myopathy (IMNM) is a subgroup of inflammatory myopathies typically presenting with proximal muscle weakness and markedly elevated muscle enzymes, mostly occurring in the setting of statin treatment. IMNM is associated with positive autoantibodies, but a subset of cases is antibody negative. Here we describe a case of myopathy occurring in association with sarcoidosis with combined features of granulomatous and necrotizing myopathy. The patient was a 54-year-old African American male with medical history significant for statin use 3 years ago, which was discontinued due to myalgia and elevated muscle enzymes and biopsy-proven sarcoidosis diagnosed on a pulmonary lymph node biopsy. He presented with progressive worsening of bilateral proximal weakness involving the upper and lower extremities. Electromyogram showed features of active myopathy with no evidence of peripheral neuropathy. Myositis panel was negative for the following antibodies: anti-Jo1, Mi-2, anti-Ku, PL-7, PL-12, OJ, EJ, and SRP. However, there was elevation of aldolase, CRP, and CK-MB. Biopsy of thigh and deltoid muscle showed necrotic muscle fibers, myophagocytosis with associated minimal inflammation, and multiple well-formed nonnecrotizing granulomas with multinucleated giant cells. Myopathic features include increased internalized nuclei, round atrophic fibers, and scattered split fibers. Specific features of IBM or DM were not present. Conclusion Myopathies developing or worsening after discontinuation of statin are rare. The association of necrotizing myopathy with sarcoidosis is not well described in the literature. Additional studies are warranted to elucidate this association.

scholarly journals Series of clinical cases of patients with idiopathic inflammatory myopathy and interstitial lung disease from the registry of idiopathic inflammatory myopathies of the Argentine Society of Rheumatology

2020 ◽  
pp. 12-17
Author(s):  
Y.M. Ponce ◽  
M.M. Zalazar ◽  
A.D. García Coello ◽  
O.L. Rillo
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Clinical Manifestations ◽  
Idiopathic Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Disease Prognosis ◽  
Immune Mediated ◽  
Necrotizing Myositis

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TI-F1γ respectively).

Diabetes and Vascular Disease: Is It All About Glycemia?

2019 ◽  
Vol 25 (29) ◽  
pp. 3112-3127 ◽  
Author(s):  
Alessandra Vecchié ◽  
Fabrizio Montecucco ◽  
Federico Carbone ◽  
Franco Dallegri ◽  
Aldo Bonaventura
Keyword(s):  
Vascular Disease ◽  
Inflammatory Cell ◽  
Vascular Dysfunction ◽  
Microvascular Complications ◽  
Coagulation Cascade ◽  
Inflammatory Cell Infiltrate ◽  
Extracellular Traps ◽  
Thrombotic Complications ◽  
Independent Entity ◽  
Complications And Mortality

Background: Diabetes is increasing over time, mainly driven by obesity, aging, and urbanization. Classical macro- and microvascular complications represent the final result of a complex interplay involving atherosclerosis at all stages. Methods: In this review, we aim at focusing on current updates in the pathophysiology of vascular disease in diabetes and discussing how new therapies might influence the management of these patients at high cardiovascular risk. Diabetes shows accelerated atherosclerosis with a larger inflammatory cell infiltrate, thus favoring the development of heart failure. ‘Diabetic cardiomyopathy’ perfectly describes a specific ischemia- and hypertension- independent entity due to diabetes-related metabolic alterations on myocardial function. Moreover, platelets from subjects with diabetes display a typical hyperreactivity explaining the stronger adhesion, activation, and aggregation. Additionally, diabetes provokes an exaggerated stimulation of the endothelium, with an increased release of reactive oxygen species and a reduced release of nitric oxide, both key elements of the endothelial dysfunction. Also, the coagulation cascade and leukocytes activate contributing to this pro-thrombotic environment. Neutrophils have been recently recognized to play a pivotal role by releasing neutrophil extracellular traps. Finally, microparticles from platelets, neutrophils or monocytes are detrimental effectors on the vessel wall and are involved both in vascular dysfunction and in thrombotic complications. Conclusion: In light of these findings, the therapeutic management of diabetes needs to be mostly focused on limiting the progression of complications by targeting precise pathophysiological mechanisms rather than the mere glycemic control, which failed to markedly reduce the risk for macrovascular complications and mortality.

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