Successful treatment of renal AA amyloidosis in familial Mediterranean fever with pegylated alpha-2a interferon

Renal AA amyloidosis is a severe consequence of chronic inflammatory diseases such as familial Mediterranean fever (FMF). FMF is caused by mutations in the MEFV gene, resulting in defective control of granulocyte-mediated inflammation. Interferon-alpha is known to induce MEFV expression in monocytes...

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Bibliographic Details
Published in:	Clinical nephrology Vol. 75 Suppl 1; p. 1
Main Authors:	Vandecasteele, S J, De Paepe, P, De Vriese, A S
Format:	Journal Article
Language:	English
Published:	Germany 01-02-2011
Subjects:	Amyloidosis - drug therapy Amyloidosis - etiology Amyloidosis - pathology Biopsy Colchicine - therapeutic use Cytoskeletal Proteins - genetics Drug Resistance Familial Mediterranean Fever - complications Familial Mediterranean Fever - drug therapy Familial Mediterranean Fever - genetics Female Humans Immunologic Factors - therapeutic use Interferon alpha-2 Interferon-alpha - therapeutic use Kidney Diseases - drug therapy Kidney Diseases - etiology Kidney Diseases - pathology Middle Aged Mutation Polyethylene Glycols - therapeutic use Pyrin Recombinant Proteins Treatment Outcome
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Summary:	Renal AA amyloidosis is a severe consequence of chronic inflammatory diseases such as familial Mediterranean fever (FMF). FMF is caused by mutations in the MEFV gene, resulting in defective control of granulocyte-mediated inflammation. Interferon-alpha is known to induce MEFV expression in monocytes and granulocytes in vitro. We present the first case of colchicine-resistant FMF in which a durable disease remission and regression of renal amyloidosis was induced by chronic treatment with pegylated interferon-alpha.
ISSN:	0301-0430
DOI:	10.5414/CN106447