Inflammatory myopathies: shedding light on promising agents and combination therapies in clinical trials

Zeng, Rachel; Glaubitz, Stefanie; Schmidt, Jens

doi:10.1080/13543784.2021.2003776

Cited by 4 publications

(3 citation statements)

References 101 publications

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“…The recent classification of these conditions incorporates clinical and pathological, and immunologic markers. Currently, autoimmune myositis is categorized into several subtypes, including dermatomyositis, inclusion body myositis (IBM), immune-mediated necrotizing myopathy (IMNM), antisynthetase syndrome (ASS) and overlap myositis [24][25][26]. The clinical presentation often involves proximal muscle weakness, such as difficulty climbing stairs or raising both arms, accompanied by dermatologic and pulmonary manifestations.…”

Section: Autoimmune Myositismentioning

confidence: 99%

Myopathies in Clinical Care: A Focus on Treatable Causes

C. Uy,

L. Rosales,

Khadilkar

2024

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Myopathies present a wide range of clinical symptoms that affect the skeletal muscles, including weakness, fatigue, and pain. While acquired myopathies receive significant attention due to the availability of treatment options, it is important to note that some inherited myopathies can also be effectively managed. These myopathies can be classified based on their underlying causes, such as infectious agents, autoimmune disorders leading to muscle inflammation, granulomatous inflammation, metabolic abnormalities within the muscle cells, skeletal muscle channel dysfunctions, prolonged ICU stay, and inherited conditions such as Duchenne muscular dystrophy. In this review, we initially present a clinical approach to neuromuscular diseases and subsequently place specific emphasis on myopathies, particularly to those that have treatment options available.

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Section: Autoimmune Myositismentioning

confidence: 99%

Myopathies in Clinical Care: A Focus on Treatable Causes

C. Uy,

L. Rosales,

Khadilkar

2024

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“…Eine Reihe an klinischen Studien wird derzeit auf internationaler Ebene und auch mit deutscher Beteiligung durchgeführt zu praktisch allen Formen der Myositis. Es handelt sich um alle Stufen der Studien, von explorativ bis zur Zulassungsstudie [ 18 , 22 , 23 ].…”

Section: Therapiestandards Und Studienausblickunclassified

Myositis: von der Diagnose zur Therapie

Schmidt

Müller‐Felber

2023

Nervenarzt

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Hintergrund Entzündliche Erkrankungen der Muskulatur sind wichtige, zum Teil schwer verlaufende Erkrankungen, die oft zu einer erheblichen Beeinträchtigung der Lebensqualität führen. Neben der Muskelschwäche gibt es oft eine Mitbeteiligung anderer Organe wie Herz, Lunge oder Speiseröhre mit Symptomen wie Atemnot oder Schluckstörung. Fragestellung Nur durch eine frühe und zuverlässige Diagnosestellung, die den aktuellen nationalen und internationalen Standards entspricht, ist eine schnelle und effektive Therapie möglich. Methoden Zum diagnostischen Repertoire gehören u. a. Autoantikörperbestimmung, Bildgebung, Muskelbiopsie, Erfassung extramuskulärer Manifestationen z. B. durch eine hochauflösende Lungencomputertomographie und eine individualisierte Tumorsuche. Nur durch eine gute Kooperation von Fächern wie Neurologie bzw. Pädiatrie, Rheumatologie, Dermatologie, Neuropathologie sowie Pulmonologie oder Kardiologie ist eine optimale Therapie und die Vermeidung irreversibler Schäden wie etwa ein Verlust der Gehfähigkeit möglich. Ergebnisse Neben der Standardimmunsuppression mit Glukokortikosteroiden, Azathioprin oder Methotrexat ist eine Eskalationstherapie mit Rituximab mittlerweile sehr gut etabliert. Zur interdisziplinären Therapie gehört die Vorstellung an einem entsprechend qualifizierten Zentrum und die Orientierung an nationalen und internationalen Standards wie Leitlinien zur Myositis. Diskussion Als hilfreiche Ressource steht das MYOSITIS NETZ e. V. ( www.myositis-netz.de ) und die International Myositis Society (iMyoS; www.imyos.org ) zur Verfügung.

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“…For non-responders or in case of severe extramuscular manifestations, second-line agents include cyclosporine and tacrolimus, which have shown beneficial effects in several studies in myositis patients [ 14 ]. Cyclophosphamide, another classical immunosuppressant, is reserved for treatment escalation in severe cases of myositis or systemic organ involvement, often in combination with other immunomodulatory agents, e.g.…”

Section: Current Treatment For Myositismentioning

confidence: 99%

Antibody Therapies in Autoimmune Inflammatory Myopathies: Promising Treatment Options

2022

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Inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), antisynthetase syndrome (ASS) and overlap myositis (OM), in short myositis, are rare diseases. All forms of myositis have progressive muscle weakness in common, with each subtype characterized by different autoantibody profiles, histological findings and extramuscular manifestations. Due to better understanding of the pathogenesis of the muscle inflammation in myositis, new molecular pathways for targeted therapy have been discovered. Current therapies aim at different components of the innate or the adaptive immune response. Additionally, non-inflammatory mechanisms in myositis have come into focus as possible treatment targets. The use of therapeutical antibodies in myositis has been examined in various clinical studies, several of them randomized controlled ones: Depletion of B-cells by rituximab has been established as treatment of refractory myositis. IVIG, an antibody therapy in the wider sense, has now been licensed for DM following a recent positive clinical trial. Negative study results were reported in randomized trials with infliximab, sifalimumab and bimagrumab. Studies on basiliximab and eculizumab are currently underway, and are expected to yield results in a couple of years. Despite some promising results of clinical studies with antibody therapy in myositis, further research is crucial to optimize the treatment for this debilitating disease and to find treatment alternatives for treatment-refractory patients.

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Inflammatory myopathies: shedding light on promising agents and combination therapies in clinical trials

Cited by 4 publications

References 101 publications

Myopathies in Clinical Care: A Focus on Treatable Causes

Myopathies in Clinical Care: A Focus on Treatable Causes

Myositis: von der Diagnose zur Therapie

Antibody Therapies in Autoimmune Inflammatory Myopathies: Promising Treatment Options

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