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Current Rheumatology Reports Mar 2022We discuss the evidence behind the use of glucocorticoids in myositis including a discussion of mechanism of action, dosing, tapering, and duration of therapy as well as... (Review)
Review
PURPOSE OF THE REVIEW
We discuss the evidence behind the use of glucocorticoids in myositis including a discussion of mechanism of action, dosing, tapering, and duration of therapy as well as glucocorticoid-related adverse events that are particularly important in myositis patients.
RECENT FINDINGS
Studies showing reduction in mortality rates after the use of glucocorticoids, better outcomes in patients treated with glucocorticoids compared to those who did not, and reduction of inflammation in muscle biopsies provide low level evidence to support use of glucocorticoids in myositis. Early initiation of therapy is associated with better functional outcomes. Use of intravenous methylprednisolone in patients with severe disease may lead to quicker recovery and reduction in long-term glucocorticoid exposure. Steroid-related myopathy and osteoporosis are glucocorticoid side effects that are particularly relevant in myositis. The optimal dose and duration of glucocorticoid therapy in myositis currently remain elusive, and this review emphasizes the need for better quality studies in this area.
Topics: Glucocorticoids; Humans; Myositis; Osteoporosis
PubMed: 35275363
DOI: 10.1007/s11926-022-01060-y -
Clinical and Experimental Rheumatology Feb 2024Inclusion body myositis (IBM) is a progressive, debilitating muscle disease commonly encountered in patients over the age of 50. IBM typically presents with asymmetric,... (Review)
Review
Inclusion body myositis (IBM) is a progressive, debilitating muscle disease commonly encountered in patients over the age of 50. IBM typically presents with asymmetric, painless, progressive weakness and atrophy of deep finger flexors and/or quadriceps muscle. Many patients with IBM develop dysphagia. However, atypical presentations of IBM with isolated dysphagia, asymptomatic hyper-CKemia, foot drop, proximal weakness, axial weakness, and facial diplegia have been reported. Other acquired and some inherited disorders may present similar to IBM, and this list gets more expansive when considering atypical presentations. In general, disease progression of IBM leads to loss of hand function and impaired ambulation, and most IBM patients become wheelchair dependent within 13-15 years of disease onset. Hence, IBM impacts negatively patients' quality of life and reduces longevity compared to the general population. Acknowledging the complete clinical spectrum of IBM presentation and excluding mimics would shorten the time to diagnosis, lead to prompt initiation of supportive management and avoid unproven therapy. Ongoing advanced phase studies in IBM provide hope that a therapy may soon be available. Therefore, an added potential benefit of early diagnosis would be prompt initiation of disease-modifying therapy once available.
Topics: Humans; Myositis, Inclusion Body; Deglutition Disorders; Quality of Life; Muscle Weakness; Myositis
PubMed: 38436356
DOI: 10.55563/clinexprheumatol/fhrx3q -
The New England Journal of Medicine Feb 2021
Topics: Adult; Creatine Kinase; Fingers; Humans; Lung; Lung Diseases, Interstitial; Male; Muscle Weakness; Myositis
PubMed: 33567195
DOI: 10.1056/NEJMicm2026773 -
Rheumatology International Jul 2023Idiopathic inflammatory myopathies (IIM) are rare disorders characterised by the presence of skeletal muscle inflammation, with interstitial lung disease (ILD) being the... (Review)
Review
Idiopathic inflammatory myopathies (IIM) are rare disorders characterised by the presence of skeletal muscle inflammation, with interstitial lung disease (ILD) being the most frequent pulmonary manifestation. The spectrum of clinical presentations of myositis related ILD (M-ILD) encompasses a chronic process to a rapidly progressive ILD (RP-ILD); which is associated with a high mortality rate. The most effective treatments remain controversial and poses a unique challenge to both rheumatologists and respiratory physicians to manage. Given the rare heterogenous nature of M-ILD, there is a paucity of data to guide treatment. The cornerstone of existing treatments encompasses combinations of immunosuppressive therapies, as well as non-pharmacological therapies. In this review, we aim to summarize the current pharmacological therapies (including its dosing regimens and side effects profiles) and non-pharmacological therapies. Based on the existing literature to date, we propose a treatment algorithm for both chronic M-ILD and RP-ILD.
Topics: Humans; Myositis; Lung Diseases, Interstitial; Lung; Inflammation; Treatment Outcome; Autoantibodies; Retrospective Studies
PubMed: 37126103
DOI: 10.1007/s00296-023-05336-z -
Current Opinion in Rheumatology Nov 2019The present review describes the interferon (IFN)-signature currently emerging as a tool for the diagnosis of idiopathic inflammatory myopathies (IIMs), and aims at... (Review)
Review
PURPOSE OF REVIEW
The present review describes the interferon (IFN)-signature currently emerging as a tool for the diagnosis of idiopathic inflammatory myopathies (IIMs), and aims at presenting the interests and limitations of this recent tool for the clinics and the research.
RECENT FINDINGS
Recent in-vivo and in-vitro transcriptomic studies have evidenced the involvement of IFNs in the pathogenesis of IIMs. A correlation between the IFN-signature and the clinical severity of IIMs has been established. Moreover, studies pointed out differences in the IFN-signature regarding the IIM subgroup (dermatomyositis, polymyositis, inclusion body myositis, anti-synthetase syndrome, immuno-mediated necrotizing myopathies), raising the hypothesis of several pathogenic processes in IIMs.
SUMMARY
IIM pathogenesis remains partially understood. IFN-signature represents one of the main recent advances in the field. IFN-signature was identified thanks to transcriptomic analyses of tissues or cells from IIM patients (muscle, skin, blood cells, muscle cells) and should allow to establish new diagnosis and better monitoring of IIM patients. It also provides a tool for investigation of IIM pathogenesis. Nevertheless, IFN-signature still requires accurate definition in order to standardize its use, notably in the clinical practice.
Topics: Gene Expression Profiling; Humans; Interferons; Myositis
PubMed: 31464706
DOI: 10.1097/BOR.0000000000000653 -
Expert Review of Clinical Immunology Feb 2023The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, the anti-synthetase syndromes, immune-mediated necrotizing myopathy and... (Review)
Review
INTRODUCTION
The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, the anti-synthetase syndromes, immune-mediated necrotizing myopathy and inclusion body myositis. However, there are uncommon localized forms that are less known. In this review, we aimed to cover these uncommon forms.
AREAS COVERED
We identified rare forms of localized myositis on the basis of list provided by the homepage of the Neuromuscular disease center of Washington University, USA and on the basis of the authors' knowledge. We searched PubMed® for relevant articles on these forms with the aim of providing as much as possible information on their clinical manifestations as well as guidance on their work-up and treatment.
EXPERT OPINION
herein, we provide un updated description of rare forms of localized myositis. These forms are often difficult to diagnose because of their localized nature and are sometimes misdiagnosed as tumors. Knowledge about these rare forms of localized myositis can aid in their recognition and treatment.
Topics: Humans; Myositis; Polymyositis; Myositis, Inclusion Body; Autoimmune Diseases; Syndrome; Autoantibodies
PubMed: 36469645
DOI: 10.1080/1744666X.2023.2154655 -
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 Pharmaceutical Design 2022Inflammatory myopathies, in short, myositis, are heterogeneous disorders that are characterized by inflammation of skeletal muscle and weakness of arms and legs.... (Review)
Review
Inflammatory myopathies, in short, myositis, are heterogeneous disorders that are characterized by inflammation of skeletal muscle and weakness of arms and legs. Research over the past few years has led to a new understanding regarding the pathogenesis of myositis. The new insights include different pathways of the innate and adaptive immune response during the pathogenesis of myositis. The importance of non-inflammatory mechanisms such as cell stress and impaired autophagy has been recently described. New target-specific drugs for myositis have been developed and are currently being tested in clinical trials. In this review, we discuss the mechanisms of action of pharmacological standards in myositis and provide an outlook of future treatment approaches.
Topics: Dermatomyositis; Humans; Inflammation; Muscle, Skeletal; Myositis; Myositis, Inclusion Body
PubMed: 34781868
DOI: 10.2174/1381612827666211115165353 -
Muscle & Nerve Mar 2023Non-necrotizing granulomatous inflammation is a rare but easily recognized histopathological finding in skeletal muscle biopsy. A limited number of diseases are known to... (Review)
Review
Non-necrotizing granulomatous inflammation is a rare but easily recognized histopathological finding in skeletal muscle biopsy. A limited number of diseases are known to be associated with non-necrotizing granulomatous myopathy. Once identified, a careful evaluation for evidence of extramuscular granulomatosis and other signs suggestive of sarcoidosis is warranted as about half of the patients have sarcoid myopathy. In addition, the presence of granulomatous myopathy should trigger a search for clinical and pathological clues of inclusion body myositis (IBM), which accounts for most of the remaining patients and can coexist with sarcoidosis. Recognizing the features of IBM in patients with granulomatous myopathy can potentially spare the patients from unnecessary exposure to immunosuppressive therapies. In patients whose granulomatous myopathy remain unexplained, further investigations should aim at identifying myasthenia gravis and other autoimmune disorders, especially those known to cause granulomatous inflammation in other organs. Laboratory investigations should include acetylcholine receptor, antimitochondrial, antineutrophil cytoplasmic, thyroglobulin, and thyroid peroxidase autoantibodies. In the appropriate clinical context, exposure to immune checkpoint inhibitors and chronic graft-vs-host disease can be causes of granulomatous myopathy. In cases of unexplained granulomatous myopathy, natural killer/T-cell lymphoma should be considered and careful histopathological examination for atypical cells and appropriate immunostaining is crucial. Identifying the etiology of granulomatous myopathy in each patient can guide appropriate treatment.
Topics: Humans; Sarcoidosis; Myositis; Muscle, Skeletal; Myositis, Inclusion Body; Granuloma; Inflammation
PubMed: 36352751
DOI: 10.1002/mus.27741 -
Cancer Immunology, Immunotherapy : CII Apr 2022Among diverse neurological immune-related adverse events (irAEs), autoimmune encephalitis, aseptic meningitis, Guillain-Barré syndrome (GBS), myasthenia gravis (MG),... (Review)
Review
Among diverse neurological immune-related adverse events (irAEs), autoimmune encephalitis, aseptic meningitis, Guillain-Barré syndrome (GBS), myasthenia gravis (MG), and myositis are particularly important. The clinical presentation may be different from that of patients with conditions unrelated to immune checkpoint inhibitors (ICIs). Many of the autoantibodies detected in patients' sera are committed to the pathogenesis, while the clinical significance of such autoantibodies in cases of neurological irAEs is different from the significance in cases of typical neuronal disorders. A broad range of clinical symptoms complicates the diagnosis of autoimmune encephalitis. The clinical features of aseptic meningitis induced by classical drugs are different from those of aseptic meningitis induced by ICIs. Although autoantibodies against synaptic receptors or neuronal cell surface proteins are not detected, anti-Ma2 antibodies, which are onconeural antibodies against intracellular proteins, are detected in patients with autoimmune encephalitis associated with ICIs. GBS induced by ICIs sometimes shows gradual progression and a relapse of symptoms, suggesting chronic inflammatory demyelinating polyneuropathy. Bulbar symptoms and myasthenic crisis are frequently observed in ICI-induced MG. Anti-acetylcholine receptor antibodies are found in only half of patients with MG occurring as an irAE. ICI-induced myositis is accompanied by ocular muscle symptoms, such as ptosis and diplopia, which can suggest MG. Patients receiving ICI treatment present clinical features and laboratory findings that represent a mixture of MG and myositis. Anti-striational antibodies may act as biomarkers in cases in which MG and myositis overlap. A correct understanding of neurological adverse events is required to achieve the best management of patients.
Topics: Autoantibodies; Humans; Immune Checkpoint Inhibitors; Myositis; Neoplasms; Nervous System Diseases
PubMed: 34515815
DOI: 10.1007/s00262-021-03053-9