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成人斯蒂尔病和慢性复发性多灶性骨髓炎:自身炎症的一种迄今尚未描述的表现。

Adult-onset Still's disease and chronic recurrent multifocal osteomyelitis: a hitherto undescribed manifestation of autoinflammation.

机构信息

Department of Medicine 3, University Erlangen-Nuremberg, Krankenhausstr. 12, 91054, Erlangen, Germany.

出版信息

Rheumatol Int. 2012 Jun;32(6):1827-9. doi: 10.1007/s00296-011-2020-x. Epub 2011 Jul 19.

DOI:10.1007/s00296-011-2020-x
PMID:21769488
Abstract

Still's disease and chronic recurrent multifocal osteomyelitis (CRMO) are febrile rheumatic diseases of unknown etiology, which predominantly affect children but can also have their initial manifestation in adults. Both can present as intermittent, relapsing episodes and are considered potential candidates within the expanding spectrum of autoinflammatory disorders, although no genetic abnormalities have been described for either of them. Here, we describe a man with an initial manifestation of abacterial multifocal osteitis at the age of 41. During a relapsing-remitting course of his illness, he increasingly developed symptoms of adult-onset Still's disease (AOSD), and the diagnosis was established according to the Yamaguchi criteria. When treated with anakinra, not only the acute symptoms disappeared promptly, but also the osteitis went into complete remission. This is to our knowledge the first description of a simultaneous occurrence of these two manifestations of autoinflammation in adulthood.

摘要

斯蒂尔病和慢性复发性多灶性骨髓炎(CRMO)是病因不明的发热性风湿性疾病,主要影响儿童,但也可在成人中首次表现出来。两者均可表现为间歇性、复发性发作,被认为是自身炎症性疾病不断扩大谱中的潜在候选疾病,尽管尚未描述这两种疾病的任何遗传异常。在这里,我们描述了一名 41 岁男性,最初表现为细菌性多灶性骨炎。在疾病反复发作的过程中,他逐渐出现成人斯蒂尔病(AOSD)的症状,并根据 Yamaguchi 标准确诊。用 anakinra 治疗后,不仅急性症状迅速消失,而且骨炎也完全缓解。据我们所知,这是首次在成人中同时出现这两种自身炎症表现的描述。

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