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- W2009059984 abstract "Sporadic inclusion body myositis (sIBM) is the most common myopathy in older patients. An effective therapy is not available to halt the slowly progressive loss of ambulation. The complex pathology includes degenerative mechanisms with an accumulation of aberrant molecules, most of all β-amyloid. At the same time, a significant inflammation with an infiltration by cytotoxic T-cells occurs in the muscle. This review summarises recent work on different pathomechanisms of sIBM. It has been demonstrated that macroautophagy is involved in the processing of β-amyloid and related to antigen-presenting mechanisms. In view of the infiltration by cytotoxic T-cells, there is evidence of a relevant antigen presentation via so-called „non-classical” co-stimulatory molecules. Moreover, in sIBM there is a specific interrelationship between degeneration-associated molecules and inflammation in the muscle. In line with this, an accumulation of β-amyloid can be induced by IL-1β in muscle cells. Taken together, these studies demonstrate that inflammatory mechanisms are of crucial relevance to the pathology of sIBM. Current therapeutic strategies include an antibody-mediated depletion of T-cells (alemtuzumab) and B-cells (rituximab). In the future, blockade of inflammatory molecules such as IL-1β and CXCL-9 as well as of β-amyloid-associated mechanisms (BACE1, autophagy) should be taken into account." @default.
- W2009059984 created "2016-06-24" @default.
- W2009059984 creator A5080901411 @default.
- W2009059984 date "2009-03-17" @default.
- W2009059984 modified "2023-09-23" @default.
- W2009059984 title "Pathogenese der Einschlusskörperchenmyositis: Stellenwert der Entzündung und Bedeutung für Therapiestrategien" @default.
- W2009059984 doi "https://doi.org/10.1055/s-0028-1090214" @default.
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