Abstract
Background
Immune-mediated necrotizing myopathy (IMNM) is a newly identified subgroup of idiopathic inflammatory myopathies. It is defined as a rare and severe disease, with symmetrical and proximal muscle weakness and a characteristic histology. An autoimmune aspect of IMNM is suggested by its association with autoantibodies directed against signal recognition particle (SRP) and 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) in the majority of patients. Statin use is strongly associated with anti-HMGCR-positive IMNM. The pathophysiological mechanisms of this disease are still poorly understood, and as a result, no therapeutic strategy has been validated to date.
Objective
The aim of this article is to provide an overview of the current knowledge about epidemiology, clinical features, and pathophysiology of IMNM, as well as treatment strategies.
Results and conclusion
IMNM is a subject of widespread interest, with quick and meaningful advances being made. In recent years, huge progress has been made in terms of diagnosis and patient management. However, the understanding of pathophysiological mechanisms and treatment strategies still requires further investigation.
Zusammenfassung
Hintergrund
Immunvermittelte nekrotisierende Myopathie (IMNM) ist eine neu identifizierte Untergruppe der idiopathischen entzündlichen Myopathien. Sie ist als seltene und schwere Erkrankung mit symmetrischer und proximaler Muskelschwäche und einer charakteristischen Histologie definiert. Auf einen Autoimmunaspekt von IMNM weist ihre Verbindung mit Autoantikörpern hin, die sich bei der Mehrheit der Patienten gegen die Signalerkennungspartikel (SRP) und 3-Hydroxy-3-Methylglutaryl-Coenzym-A-Reduktase (HMGCR) richten. Statingebrauch steht in enger Verbindung mit Anti-HMGCR-positiver IMNM. Das Verständnis der pathophysiologischen Mechanismen dieser Erkrankung ist bislang nur dürftig , weshalb bis jetzt keine therapeutische Strategie anerkannt ist.
Ziel
Das Ziel dieses Artikels ist es, einen Überblick über das derzeitige Wissen hinsichtlich Epidemiologie, klinischer Merkmale und der Pathophysiologie von IMNM sowie zu Behandlungsstrategien zu liefern.
Ergebnisse und Schlussfolgerung
IMNM ist Gegenstand breiten Interesses, wobei schnelle und bedeutsame Fortschritte gemacht werden. In den vergangenen Jahren gab es enorme Entwicklungen bei der Diagnose und im Patientenmanagement. Das Verständnis der pathophysiologischen Mechanismen und Behandlungsstrategien erfordert allerdings weitere Forschung.
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C. Bergua, H. Chiavelli, J.P. Simon, O. Boyer, F. Jouen, W. Stenzel, and J. Martinet state that there are no conflicts of interest.
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Bergua, C., Chiavelli, H., Simon, J.P. et al. Immune-mediated necrotizing myopathy. Z Rheumatol 75, 151–156 (2016). https://doi.org/10.1007/s00393-015-0029-3
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DOI: https://doi.org/10.1007/s00393-015-0029-3