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Rheumatic Diseases Clinics of North... Nov 2019Major advances have been made in the field of idiopathic inflammatory myopathies (IIM), or myositis, that are likely to facilitate development of new therapeutic... (Review)
Review
Major advances have been made in the field of idiopathic inflammatory myopathies (IIM), or myositis, that are likely to facilitate development of new therapeutic strategies that have not yet been applied in this group of diseases. These advances include new classification criteria to better identify the patients with IIM, detection of several new myositis-specific autoantibodies that facilitates subgrouping of patients into more specific clinical phenotypes, development of outcome measures for disease activity, and new response criteria. We have learned from clinical studies that exercise is an important part of treatment and that pharmacologic treatment should be combined with exercise.
Topics: Antirheumatic Agents; Autoantibodies; Humans; Myositis; Patient Care Management; Severity of Illness Index; Symptom Assessment
PubMed: 31564297
DOI: 10.1016/j.rdc.2019.07.006 -
Frontiers in Immunology 2023Idiopathic inflammatory myopathies (IIMs) are common autoimmune diseases that affect skeletal muscle quality and function. The lack of an early diagnosis and treatment... (Review)
Review
Idiopathic inflammatory myopathies (IIMs) are common autoimmune diseases that affect skeletal muscle quality and function. The lack of an early diagnosis and treatment can lead to irreversible muscle damage. Non-coding RNAs (ncRNAs) play an important role in inflammatory transfer, muscle regeneration, differentiation, and regulation of specific antibody levels and pain in IIMs. ncRNAs can be detected in blood and hair; therefore, ncRNAs detection has great potential for diagnosing, preventing, and treating IIMs in conjunction with other methods. However, the specific roles and mechanisms underlying the regulation of IIMs and their subtypes remain unclear. Here, we review the mechanisms by which micro RNAs and long non-coding RNA-messenger RNA networks regulate IIMs to provide a basis for ncRNAs use as diagnostic tools and therapeutic targets for IIMs.
Topics: Humans; Myositis; RNA, Untranslated; Autoimmune Diseases; MicroRNAs; Muscle, Skeletal
PubMed: 37744337
DOI: 10.3389/fimmu.2023.1227945 -
Arthritis & Rheumatology (Hoboken, N.J.) Feb 2023Idiopathic inflammatory myopathies (IIMs) comprise a heterogeneous group of rare immune-mediated disorders that primarily affect muscles but also lead to dysfunction in... (Review)
Review
Idiopathic inflammatory myopathies (IIMs) comprise a heterogeneous group of rare immune-mediated disorders that primarily affect muscles but also lead to dysfunction in other organs. Five different clinical subphenotypes of IIM have been distinguished: dermatomyositis, polymyositis, inclusion body myositis, antisynthetase syndrome, and immune-mediated necrotizing myopathy. Excess mortality and morbidity associated with IIM are largely attributed to comorbidities, particularly cancer. The risk of malignancy is not equally distributed among IIM groups and is particularly high among patients with dermatomyositis. The cancer risk peaks around 3 years on either side of the IIM diagnosis and remains elevated even 10 years after the onset of the disease. Lung, colorectal, and ovarian neoplasms typically arise before the onset of IIM, whereas melanoma, cervical, oropharyngeal, and nonmelanoma skin cancers usually develop after IIM diagnosis. Given the close temporal proximity between IIM diagnosis and the emergence of malignancy, it has been proposed that IIM could be a consequence rather than a cause of cancer, a process known as a paramalignant phenomenon. Thus, a separate group of IIMs related to paramalignant phenomenon has been distinguished, known as cancer-associated myositis (CAM). Although the relationship between IIM and cancer is widely recognized, the pathophysiology of CAM remains elusive. Given that genetic factors play a role in the development of IIM, dissection of the molecular mechanisms shared between IIM and cancer presents an opportunity to examine the role of autoimmunity in cancer development and progression. In this review, the evidence supporting the contribution of genetics to CAM will be discussed.
Topics: Humans; Dermatomyositis; Myositis; Polymyositis; Myositis, Inclusion Body; Melanoma
PubMed: 36053262
DOI: 10.1002/art.42345 -
Best Practice & Research. Clinical... Feb 2020The idiopathic inflammatory myopathies are a group of heterogeneous autoimmune connective tissue diseases. Despite increase in the understanding of these conditions,... (Review)
Review
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. Several conditions can mimic myositis, including metabolic myopathies, genetic myopathies and neurological disease. In addition, the heterogeneity within the idiopathic inflammatory myopathy spectrum can also create diagnostic confusion, referred to here as 'myositis chameleons'. This includes inclusion body myositis, immune-mediated necrotizing myopathy, hypomyopathic variants of anti-synthetase syndrome and overlap disease. We highlight the importance of a thorough diagnostic workup, refer to updated classification criteria and emphasize the importance of myositis autoantibody testing. Where diagnostic doubt exists, the involvement of a specialist centre and a multidisciplinary team is vital.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Myositis; Myositis, Inclusion Body
PubMed: 32063440
DOI: 10.1016/j.berh.2020.101486 -
Joint Bone Spine Nov 2022
Topics: Humans; Antibodies, Monoclonal, Humanized; Myositis
PubMed: 35636703
DOI: 10.1016/j.jbspin.2022.105414 -
Der Nervenarzt Jun 2023Inflammatory diseases of the skeletal muscle are important, often severe diseases with a considerable impact on the quality of life. In addition to muscle weakness there... (Review)
Review
BACKGROUND
Inflammatory diseases of the skeletal muscle are important, often severe diseases with a considerable impact on the quality of life. In addition to muscle weakness there is often involvement of other organs, such as the heart, lungs and esophagus with symptoms such as dyspnea or dysphagia.
PURPOSE
A fast and effective treatment is only possible by an early and reliable diagnosis according to current national and international standards.
METHODS
The diagnostic repertoire includes autoantibody testing, imaging, muscle biopsy, detection of extramuscular manifestations, e.g., by high-resolution lung computed tomography (CT) and an individualized tumor search. An optimal treatment and the avoidance of irreversible damage, such as a loss of walking ability, are only possible through a good interdisciplinary cooperation including neurology or pediatrics, rheumatology, dermatology, neuropathology, pulmonology and cardiology.
RESULTS
In addition to standard immunosuppression with glucocorticosteroids, azathioprine or methotrexate, escalation treatment with rituximab is now well established. Interdisciplinary treatment according to national and international standards, such as guidelines on myositis, should be coordinated at qualified centers of excellence.
DISCUSSION
Helpful resources are the MYOSITIS NETZ ( www.myositis-netz.de ) and the International Myositis Society (iMyoS; www.imyos.org ).
Topics: Humans; Child; Quality of Life; Myositis; Muscle, Skeletal; Autoantibodies; Treatment Outcome
PubMed: 37222759
DOI: 10.1007/s00115-023-01490-8 -
Joint Bone Spine Oct 2019Skeletal muscle inflammation is the feature shared by all forms of myositis. However, the muscle damage ranges in severity from asymptomatic to responsible for severe... (Review)
Review
Skeletal muscle inflammation is the feature shared by all forms of myositis. However, the muscle damage ranges in severity from asymptomatic to responsible for severe weakness. In addition, myositis usually occurs as a systemic disease that affects multiple organs. More specifically myositis should be considered in patients with muscular, cutaneous, pulmonary, and articular manifestations. The definitive diagnosis and classification of myositis has benefited considerably in recent years from the identification of characteristic autoantibodies. Nonetheless, a muscle biopsy is often necessary to confirm the diagnosis, and the differential diagnoses may raise challenges that require evaluation at a myositis referral center. The management depends on the type of myositis. Treatments should be provided for both the systemic complications (involving the lungs, heart, gastrointestinal tract, and/or joints) and the comorbidities (cancer and risks factors for cardiovascular disease, thromboembolism, and fractures), which together determine the prognosis. Many drugs are available for treating myositis. Findings from randomized controlled trials will help to use them optimally.
Topics: Autoantibodies; Biopsy; Guidelines as Topic; Humans; Muscle, Skeletal; Myositis
PubMed: 30711692
DOI: 10.1016/j.jbspin.2019.01.013 -
Clinical Rheumatology Oct 2023Cardiovascular involvement in idiopathic inflammatory myopathies (IIM) is an understudied area which is gaining increasing recognition in recent times. Recent advances... (Review)
Review
Cardiovascular involvement in idiopathic inflammatory myopathies (IIM) is an understudied area which is gaining increasing recognition in recent times. Recent advances in imaging modalities and biomarkers have allowed the detection of subclinical cardiovascular manifestations in IIM. However, despite the availability of these tools, the diagnostic challenges and underestimated prevalence of cardiovascular involvement in these patients remain significant. Notably, cardiovascular involvement remains one of the leading causes of mortality in patients with IIM. In this narrative literature review, we outline the prevalence and characteristics of cardiovascular involvement in IIM. Additionally, we explore investigational modalities for early detection of cardiovascular involvement, as well as newer approaches in screening to facilitate timely management. Key points • Cardiac involvement in IIM in majority cases is subclinical and a major cause of mortality. • Cardiac magnetic resonance imaging is sensitive for detection of subclinical cardiac involvement.
Topics: Humans; Myositis; Heart; Magnetic Resonance Imaging; Biomarkers
PubMed: 37148365
DOI: 10.1007/s10067-023-06599-4 -
Best Practice & Research. Clinical... Jun 2022Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases. There are distinct subgroups, including antisynthetase syndrome, dermatomyositis,... (Review)
Review
Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases. There are distinct subgroups, including antisynthetase syndrome, dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and sporadic inclusion body myositis. In patients with IIM, autoantibodies are present in up to 80% of the patients. These autoantibodies are often characterized as myositis-specific autoantibodies (MSA) or myositis-associated autoantibodies (MAA). The recognition of the importance of autoantibodies, especially MSA, is increasing in recent years. In this chapter, we provide an overview of the MSAs, including some new autoantibodies of interest as they target mainly muscle-specific autoantigen, in clinical classification, the measurement of the disease activity, and a possible role in the pathogenesis in the patients with IIM.
Topics: Autoantibodies; Autoantigens; Autoimmune Diseases; Dermatomyositis; Humans; Myositis; Myositis, Inclusion Body
PubMed: 35810122
DOI: 10.1016/j.berh.2022.101767 -
Acta Myologica : Myopathies and... Dec 2020Giant cell myositis (GCMm) and giant cell myocarditis (GCMc) are two rare autoimmune conditions. Among these, GCMc is a life-threatening disease with a 1-year mortality... (Review)
Review
Giant cell myositis (GCMm) and giant cell myocarditis (GCMc) are two rare autoimmune conditions. Among these, GCMc is a life-threatening disease with a 1-year mortality rate of 70%. Lethal ventricular arrhythmias, rapid evolution to heart failure and sudden death risk makes GCMc an emergency condition. It is thought to be mediated by T-cells and characterized by the presence of myofiber necrosis and giant cells in biopsies. Most commonly co-manifesting conditions with GCMm and/or GCMc are thymoma, myasthenia gravis and orbital myositis, all of which are treatable. As suspicion is the key approach in diagnosis, the physician following patients with thymoma with or without myasthenia gravis and with orbital myositis should always be alert. The fatal nature of GCMc associated with these relatively benign diseases deserves a special emergency attention with prompt institution of combined immunosuppressive treatment and very early inclusion of heart failure teams.
Topics: Giant Cells; Humans; Myocarditis; Myositis
PubMed: 33458585
DOI: 10.36185/2532-1900-033