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Journal of Neuromuscular Diseases 2018Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with... (Review)
Review
Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyography (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis.
Topics: Biopsy; Dermatomyositis; Disease Management; Electromyography; Humans; Magnetic Resonance Imaging; Muscle, Skeletal; Myositis; Myositis, Inclusion Body; Polymyositis
PubMed: 29865091
DOI: 10.3233/JND-180308 -
Journal of Immunoassay & Immunochemistry 2017Idiopathic inflammatory myopathies (IIM) represent a heterogeneous group of rare muscular diseases, with no clearly known causes. IIM frequently have an incomplete... (Review)
Review
Idiopathic inflammatory myopathies (IIM) represent a heterogeneous group of rare muscular diseases, with no clearly known causes. IIM frequently have an incomplete response to treatment due to the difficulty in distinguishing between IIM forms, and due to neglect their non-inflammatory causes. Important data concerning non-immune mechanisms in IIM pathology have been recently accumulated. There is a correlation between inflammatory and non-inflammatory mechanisms, but their involvement in IIM pathogenesis is still unknown. Here we review some of the most important data regarding the non-immune IIM pathology, highlighting possible future therapeutic targets: endoplasmic reticulum stress, ATP metabolism, ROS generation, autophagy, and microRNAs disturbances.
Topics: Autophagy; Endoplasmic Reticulum Stress; Humans; MicroRNAs; Myositis; Reactive Oxygen Species
PubMed: 28375073
DOI: 10.1080/15321819.2017.1298525 -
Rheumatic Diseases Clinics of North... May 2024Myositis induced by immune checkpoint inhibitors (ICIs) is an infrequent, potentially fatal, immune-related adverse event. It has higher incidence in patients who... (Review)
Review
Myositis induced by immune checkpoint inhibitors (ICIs) is an infrequent, potentially fatal, immune-related adverse event. It has higher incidence in patients who receive combination ICI therapy compared to monotherapy. Patients can present with clinical manifestation symptoms of myositis alone or in combination with myocarditis and/or myasthenia gravis, which significantly worsens the course and prognosis. Diagnosis can generally be made on the basis of clinical presentation, elevation of muscle enzymes, and electromyographic changes, but some patients may require a muscle biopsy. The first line of therapy is high-dose corticosteroids, followed by immunosuppression, plasmapheresis, or intravenous immunoglobulin in patients with severe disease.
Topics: Humans; Immune Checkpoint Inhibitors; Myositis; Immunoglobulins, Intravenous
PubMed: 38670726
DOI: 10.1016/j.rdc.2024.02.003 -
Current Opinion in Rheumatology Nov 1997Idiopathic orbital myositis is a subtype of nonspecific orbital inflammation primarily involving the extraocular muscles. It occurs most frequently in young to... (Review)
Review
Idiopathic orbital myositis is a subtype of nonspecific orbital inflammation primarily involving the extraocular muscles. It occurs most frequently in young to middle-aged adults with a 2 to 1 female predominance. The cardinal clinical feature is orbital pain exacerbated by eye movement. Other common findings include diplopia, proptosis (which is generally minimal), conjunctival injection and chemosis, and periorbital edema. Thyroid eye disease is commonly confused with orbital myositis, but the latter is characterized by a more acute onset, more severe pain, and a rapid response to systemic corticosteroid therapy. Echography and CT scanning reveal enlarged muscle bellies and thickened tendons, with low internal reflectivity echographically. Although the cause of orbital myositis is unknown, an immune-mediated pathophysiologic mechanism is likely. This review summarizes recent findings regarding the epidemiology, diagnosis, pathophysiology, and treatment of idiopathic orbital myositis.
Topics: Diagnosis, Differential; Humans; Myositis; Orbital Diseases
PubMed: 9375279
DOI: 10.1097/00002281-199711000-00005 -
Internal Medicine Journal Jun 2021Idiopathic inflammatory myopathy (IIM) is the umbrella term including dermatomyositis (DM), polymyositis (PM), overlap myositis (OM), sporadic inclusion body myositis... (Review)
Review
Idiopathic inflammatory myopathy (IIM) is the umbrella term including dermatomyositis (DM), polymyositis (PM), overlap myositis (OM), sporadic inclusion body myositis (IBM) and necrotising autoimmune myopathy (NAM), also known as immune-mediated necrotising myopathy. There is some debate as to whether PM exists as a discrete entity, or perhaps is an overly generalising term encompassing connective tissue disease associated myositis, or OM, and the previously poorly recognised NAM. As such, PM will not be covered in detail in this review. DM, OM and NAM all present similarly, with proximal weakness and elevated creatine kinase (CK) level. By contrast, IBM preferentially involves the long finger flexors and quadriceps, and presents with a normal or only mildly elevated CK. Developments in serological testing and imaging are shifting the diagnostic paradigm away from a reliance on histopathology. The therapeutic armamentarium for IIM continues to evolve, with intravenous immunoglobulin and rituximab proving to be successful for refractory disease. This review will provide a diagnostic algorithm for the clinician to help distinguish between IIM subtypes - with emphasis on clinical assessment, serology and imaging, as well as discussion of therapeutic options and escalation of immunotherapy.
Topics: Dermatomyositis; Humans; Myositis; Myositis, Inclusion Body; Polymyositis
PubMed: 34155760
DOI: 10.1111/imj.15358 -
Biomolecules Jan 2022Idiopathic inflammatory myopathies are a group of rare connective tissue diseases with a well-documented association with malignancy. The mechanisms underlying the... (Review)
Review
Idiopathic inflammatory myopathies are a group of rare connective tissue diseases with a well-documented association with malignancy. The mechanisms underlying the increased risk of neoplasms in the course of myositis are not fully understood. The Pubmed database has been thoroughly screened for articles concerning cancer-associated myositis (CAM). The article summarizes the current state of knowledge on the epidemiology and pathogenesis of CAM. Furthermore, it analyses potential risk and protective factors for developing CAM, with particular emphasis on the association with distinct serological profiles. The review summarizes recommendations proposed so far for the management of CAM and presents a novel scheme for cancer screening proposed by the authors. Moreover, promising areas requiring further research were indicated.
Topics: Early Detection of Cancer; Humans; Myositis; Neoplasms
PubMed: 35053259
DOI: 10.3390/biom12010111 -
Neuromuscular Disorders : NMD Nov 2016Amongst the heterogeneous group of inflammatory myopathies, focal myositis stands as a rare and benign dysimmune disease. Although it can be associated with root and/or... (Review)
Review
Amongst the heterogeneous group of inflammatory myopathies, focal myositis stands as a rare and benign dysimmune disease. Although it can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of focal myositis is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern. MRI reveals a contrast enhanced enlarged muscle appearing hyper-intense on FAT-SAT T2 weighted images. Adjacent structures are spared and there are no calcifications. Serum creatine kinase (CK) levels are usually moderately augmented and biological markers of systemic inflammation are absent in most cases. Pathological histological features include marked variation in fibre size, inflammatory infiltrates mostly composed of T CD4+ lymphocytes and macrophages, degenerating/regenerating fibres and interstitial fibrosis. Differential diagnoses are numerous and include myositis of other origin with focal onset. Steroid treatment should be reserved for patients who present with major pain, nerve lesions, associated autoimmune disease, or elevated C reactive protein or CK.
Topics: Humans; Myositis
PubMed: 27726926
DOI: 10.1016/j.nmd.2016.08.014 -
International Journal of Rheumatic... Nov 2023
Topics: Humans; Myositis
PubMed: 37910028
DOI: 10.1111/1756-185X.14850 -
Nature Reviews. Rheumatology Jan 2017
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Brain and Nerve = Shinkei Kenkyu No... Apr 2010Inflammatory myopathies, also referred to as myositis, are a heterogeneous group of chronic inflammatory muscle diseases characterized by various clinical features and... (Review)
Review
Inflammatory myopathies, also referred to as myositis, are a heterogeneous group of chronic inflammatory muscle diseases characterized by various clinical features and histological changes; in addition, patient with these disease exhibit positivity for autoantibodies as well as progressive inflammatory muscle damage and experienced weakness. Although it has been well known for a century that myositis, particularly dermatomyositis, can be associated with malignancy, it was not until recently that the result of robust epidemiological studies confirmed this association. Malignancy-associated myositis differs from primary myositis in many aspects. Prognosis and life expectancy for patients are determined on the basis of the underlying malignancy. Therefore, patient-specific examinations to detect an underlying cancer are important for the management of these patients. Recently, a novel myositis-specific autoantibody (anti-p155 or p155/p140 antibody) was identified in malignancy-associated myositis. The discovery of this autoantibody is important not only for an early diagnosis of adult-onset myositis patients with a higher risk of malignancy but also for a better understanding of the pathogenesis of paraneoplastic myositis. In addition, it has also been recently found that both regenerating cells in myositis muscles and several cancers known to be associated with myositis express high levels of myositis-specific autoantigens. Therefore, a model of paraneoplasia focusing on the expression of common autoantigens expression and immuno-targeting between cancer and muscle tissues in myositis has been proposed. In this review, we aim to describe epidemiological evidence for an association between myositis and malignancy, and to describe the clinical features of malignancy-associated myositis. We also aim to focus on a recently proposed model to understand the development of malignancy-associated myositis.
Topics: Autoantibodies; Autoantigens; Biomarkers; Humans; Myositis; Neoplasms; Paraneoplastic Syndromes, Nervous System; Risk Factors
PubMed: 20420184
DOI: No ID Found