Treatment and prevention of the amyloidoses: Can the lessons learned be applied to sporadic inclusion-body myositis?
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To the Editor:
Buxbaum's excellent article on amyloidoses refers to amyloidosis of familial Mediterranean fever (FMF).1 AA amyloidosis related with FMF represents a distinct entity of the disease. Although it is associated with a certain molecular background (i.e., pyrin gene mutations) and it is usually complicating a long manifested FMF, it may also develop in an otherwise asymptomatic state. It can be the presenting symptom of FMF called phenotype II. Accordingly, FMF amyloidosis may be a direct disease-associated condition rather than a complication of the disease inflammatory activity.
Recent advances in our understanding of the role of pyrin (both normal and mutated) in apoptosis and inflammation support this hypothesis. Colchicine, the drug for controlling the frequency and severity of FMF crises, may also …
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