Diagnosis, pathogenesis and treatment of myositis: recent advances

Carstens, PO; Schmidt, Jens

doi:10.1111/cei.12194

Cited by 108 publications

(111 citation statements)

References 107 publications

(131 reference statements)

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“…It has been reported that the average age of onset in SRP-correlated autoimmune necrotizing myopathy is approximately 50 years old (Carstens and Schmidt, 2014), which is in agreement with results from our present study, where peak onset age was found to be between 51-60 years of age.…”

Section: Discussionsupporting

confidence: 94%

Expression of anti-SRP19 antibody in muscle tissues from patients with autoimmune necrotizing myopathy

et al. 2016

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ABSTRACT. This study aimed to investigate the role of anti-SRP19 antibody in muscle tissues of patients with autoimmune necrotizing myopathy. Immunohistochemistry staining was used to determine the expression of anti-SRP19 antibodies in muscle tissues of autoimmune necrotizing myopathy patients. Results demonstrated that anti-SRP19 antibody was expressed in 71.4% (20/28) of muscle tissue specimens from patients with autoimmune necrotizing myopathy. Anti-SRP19 antibody expression was mainly localized in cytoplasm of necrotic muscle fibers surrounding the small blood vessels and interstitial cells. There were no significant differences in the age, course of disease, muscle, and creatine kinase levels between patients with positive or negative expression of anti-SRP19 antibodies. The expression levels of anti-SRP19, serum anti-nuclear antibodies, as well as anti-Ro-52, anti-SSA, anti-Sm, and anti-Jo-1 antibodies were not significantly different among groups. This study demonstrates that anti-SRP19 antibody is highly expressed in muscle tissues of patients with autoimmune necrotizing myopathy, and suggests that this protein may be involved in the origin and progression of the disease.

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Section: Discussionsupporting

confidence: 94%

Expression of anti-SRP19 antibody in muscle tissues from patients with autoimmune necrotizing myopathy

et al. 2016

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“…Although their efficacy has never been confirmed in a randomized controlled trial (RCT), corticosteroids are the traditional first-line treatment for DM, PM, and OS once the diagnosis has been established and other disorders that can mimic an IMIM have been excluded [6,37,38]. It is usual to start treatment with oral prednisolone at a dose of~1 mg/kg/day and it is preferable to avoid using higher doses because of the greater risk of steroid side effects.…”

Section: Conventional Therapiesmentioning

confidence: 99%

“…Previous reviews have discussed the practical aspects of managing ongoing therapy and have emphasized the importance of not continuing high doses of steroids for too long and balancing this against not tapering the dose too quickly. They advocate the introduction of an immunosuppressive agent such as methotrexate, azathioprine, or mycophenolate earlier rather than later as a steroid-sparing strategy [34,[38][39][40][41]. Oral pulse therapy with dexamethasone (monthly cycles of 40 mg/ day for 4 consecutive days) has been suggested as an alternative to prednisolone and has been shown to be as effective in a RCT and to have a more favorable side effect profile, but the time to relapse was shorter with dexamethasone [42].…”

Section: Conventional Therapiesmentioning

confidence: 99%

Immunotherapies for Immune-Mediated Myopathies: A Current Perspective

Needham

Mastaglia

2016

Neurotherapeutics

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Summary Until recently, the treatment of immune-mediated inflammatory myopathies has largely been empirical with glucocorticoids, steroid-sparing immunosuppressive drugs, and intravenous immunoglobulin. However, a proportion of patients are only partially responsive to these therapies, and there has been a need to consider alternative treatment approaches. In particular, patients with inclusion body myositis are resistant to conventional immunotherapies or show only a transient response, and remain a major challenge. With increasing recognition of the different subtypes of immune-mediated inflammatory myopathies, and improved understanding of their pathogenesis, more targeted treatments are now being trialled. The overall approach to treatment, and novel therapies targeting B cells, T cells, and specific cytokines are discussed in this review.

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“…Inclusion body myositis (IBM) is a progressive slow‐onset inflammatory myopathy that is characterized by the concomitant presence of multi‐focal myofiber‐surrounding lymphocytic infiltrates as well as vacuolar myodegeneration 1, 2, 3, 4. Together with dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myositis (NAM) and overlap myositis (OM), IBM belongs to the heterogenous group of inflammatory myopathies and, amongst individuals 50 years of age and older, it is considered as a relatively frequent disorder 3, 5.…”

Section: Introductionmentioning

confidence: 99%

Immune and myodegenerative pathomechanisms in inclusion body myositis

Keller

Schmidt

Lünemann

2017

Ann Clin Transl Neurol

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Inclusion Body Myositis (IBM) is a relatively common acquired inflammatory myopathy in patients above 50 years of age. Pathological hallmarks of IBM are intramyofiber protein inclusions and endomysial inflammation, indicating that both myodegenerative and inflammatory mechanisms contribute to its pathogenesis. Impaired protein degradation by the autophagic machinery, which regulates innate and adaptive immune responses, in skeletal muscle fibers has recently been identified as a potential key pathomechanism in IBM. Immunotherapies, which are successfully used for treating other inflammatory myopathies lack efficacy in IBM and so far no effective treatment is available. Thus, a better understanding of the mechanistic pathways underlying progressive muscle weakness and atrophy in IBM is crucial in identifying novel promising targets for therapeutic intervention. Here, we discuss recent insights into the pathomechanistic network of mutually dependent inflammatory and degenerative events during IBM.

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Diagnosis, pathogenesis and treatment of myositis: recent advances

Cited by 108 publications

References 107 publications

Expression of anti-SRP19 antibody in muscle tissues from patients with autoimmune necrotizing myopathy

Expression of anti-SRP19 antibody in muscle tissues from patients with autoimmune necrotizing myopathy

Immunotherapies for Immune-Mediated Myopathies: A Current Perspective

Immune and myodegenerative pathomechanisms in inclusion body myositis

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