Improvement of Sensoneurinal Hearing Loss in a Patient with Muckle-Wells Syndrome Treated with Anakinra

被引：14

作者：

Klein, A. K. ^{[1
]}

Horneff, G. ^{[1
]}

机构：

[1] Zentrum Allgemeine Padiatrie & Neonatol, Asklepios Kinderklin Sankt Augustin, D-53757 St Augustin, Germany

来源：

KLINISCHE PADIATRIE | 2010年 / 222卷 / 04期

关键词：

Muckle-Wells syndrome; Anakinra; sensoneurinal hearing loss; periodic fever syndromes; MUTATIONS; CIAS1; DEAFNESS; INFLAMMASOME; AMYLOIDOSIS; URTICARIA; EFFICACY;

D O I：

10.1055/s-0029-1239527

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Muckle Wells syndrome is an autoinflammatory disease in the group of cryopyrin associated periodic syndromes (CAPS). We report the case of an 8 year old girl with MWS who presented with remitting fever, urticaria, remitting coxitis, osteitis, bilateral uveitis anterior, elevated levels of C-reactive protein (CRP) and Serum amyloid A (SAA) and progressive sensoneurinal hearing loss. After starting treatment with anakinra, clinical symptoms dissolved almost completely for about two years now. CRP and SAA levels normalized quickly and sustained and as a consequence the risk of amyloidosis may be minimized. Notable is the complete recovery from sensoneurinal hearing loss merely two months after start of treatment. This brings up questions about pathophysiology of sensoneurinal hearing loss in MWS and emphasizes the benefits of an early diagnosis, as an early start of treatment possibly reduces long-term damage.

引用

页码：266 / 268

页数：3

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