摘要
家族性地中海热(FMF)是最常见的遗传性自身炎症性综合症。FMF是由编码哌啉蛋白的MEFV基因突变引起的。FMF的临床特征是时有时无的,阵发性发作的发热和浆膜炎,持续时间为1-3天。患者可能发展成肾淀粉样变性。秋水仙素阻止疾病侵袭和肾淀粉样变性。有5%到10%的FMF患者耐药或不能耐受秋水仙碱。秋水仙素耐药的病人可以接受生物治疗。抗白介素-1药物是生物治疗的最重要药物。这篇综述,将对秋水仙碱耐药和治疗选择进行阐述。
关键词: 秋水仙碱耐药,家族性地中海热,治疗
Current Medicinal Chemistry
Title:Biological Agents in Familial Mediterranean Fever Focusing on Colchicine Resistance and Amyloidosis
Volume: 22 Issue: 16
Author(s): Betul Sozeri and Ozgur Kasapcopur
Affiliation:
关键词: 秋水仙碱耐药,家族性地中海热,治疗
摘要: Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. FMF is caused by mutations in the MEFV gene which encodes the pyrin protein. FMF is characterized by sporadic, paroxysmal attacks of fever and serosal inflammation, lasting 1-3 days. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis.5% to 10% of the patients with FMF are resistant or intolerant to colchicine. Colchicine resistant patients may receive biological therapies. Anti-interleukin-1 drugs are the most important agents of biological treatments. In this review, colchicine resistance and treatment options will be evaluated.
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Cite this article as:
Betul Sozeri and Ozgur Kasapcopur , Biological Agents in Familial Mediterranean Fever Focusing on Colchicine Resistance and Amyloidosis, Current Medicinal Chemistry 2015; 22 (16) . https://dx.doi.org/10.2174/0929867322666150311152300
DOI https://dx.doi.org/10.2174/0929867322666150311152300 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
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