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[成人威斯勒-范科尼综合征和斯蒂尔病。疾病分类学和病因学问题及边缘型。附7例报告]

[Wissler-Fanconi syndrome and Still's disease in adults. Nosological and etiological problems and borderline forms. Apropos of 7 cases].

作者信息

Alcalay M, Becq-Giraudon B, Aboul Hosn H

出版信息

Sem Hop. 1983 Jan 27;59(4):233-9.

PMID:6301051
Abstract

In both Still disease and Wissler-Fanconi syndrome, onset may occur in adulthood and the clinical picture includes acute, often migratory, polyarthritis, polymorphic exanthema and a major inflammatory syndrome with fever (often intermittent) and high polymorphonuclear leukocyte counts. The grounds for this double designation are not clearly stated in the medical literature: does it refer to two separate entities or not? Some authors plainly stand for a unitary opinion. We report on seven cases in adults, five of which recovered without sequellae, while two developed respectively into rheumatoid arthritis and seronegative polyarthritis. We therefore propound a dualistic concept although, in the present state of our knowledge, there is no means of predicting at onset whether the disease will resolve or progress towards polyarthritis. We suggest that the term Still disease be used only when chronic and/or destructive arthritis develops. Among the forms which are neither chronic nor destructive, there seems to be a number of cases in allergic patients with poor tolerance of chrysotherapy. Some of these patients could recover after the cure of an often latent focal infection, usually of the upper respiratory tract, ear or oral cavity, resistant to antibiotics. In the other cases, corticosteroid therapy is usually very effective in arresting an exacerbation of the disease.

摘要

在斯蒂尔病和维勒-范科尼综合征中,发病可能发生在成年期,临床表现包括急性、通常为游走性的多关节炎、多形性皮疹以及伴有发热(常为间歇性)和高多形核白细胞计数的主要炎症综合征。医学文献中并未明确说明这种双重命名的依据:它是指两个不同的实体还是并非如此?一些作者明确支持单一观点。我们报告了7例成人病例,其中5例康复且无后遗症,而另外2例分别发展为类风湿关节炎和血清阴性多关节炎。因此,尽管就我们目前的知识水平而言,在疾病发作时无法预测其是否会缓解或发展为多关节炎,但我们提出一种二元概念。我们建议仅在慢性和/或破坏性关节炎发生时使用“斯蒂尔病”这一术语。在既非慢性也非破坏性的类型中,似乎有一些病例出现在对金疗法耐受性差的过敏患者中。这些患者中的一些人在治愈通常对抗生素耐药的、常为潜伏性的局部感染(通常为上呼吸道、耳部或口腔感染)后可能康复。在其他情况下,皮质类固醇疗法通常对阻止疾病加重非常有效。

相似文献

1
[Wissler-Fanconi syndrome and Still's disease in adults. Nosological and etiological problems and borderline forms. Apropos of 7 cases].[成人威斯勒-范科尼综合征和斯蒂尔病。疾病分类学和病因学问题及边缘型。附7例报告]
Sem Hop. 1983 Jan 27;59(4):233-9.
2
[Still's disease in adults and Wissler-Fanconi syndrome].
Rev Rhum Mal Osteoartic. 1973 Jan;40(1):1-8.
3
A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?五名青少年出现瘙痒性线状荨麻疹皮疹、发热及全身炎症性疾病:成人斯蒂尔病还是无关节炎的系统性幼年特发性关节炎?
Pediatr Dermatol. 2004 Sep-Oct;21(5):580-8. doi: 10.1111/j.0736-8046.2004.21513.x.
4
[Still's disease in adults: apropos of 6 cases].
Schweiz Rundsch Med Prax. 1989 May 23;78(21):602-8.
5
[Adult-onset Still's disease].[成人斯蒂尔病]
Ann Acad Med Stetin. 2006;52 Suppl 2:111-4.
6
[Clinical study of Still's disease as a distinct disease entity].[作为一种独特疾病实体的斯蒂尔病的临床研究]
Ryumachi. 1993 Oct;33(5):410-5.
7
[Wissler - Fanconi syndrome or Still's disease?].
Wiad Lek. 1976 Jan 1;29(1):35-9.
8
Still's arthritis in adults: disease or syndrome?
J Rheumatol. 1983 Oct;10(5):758-62.
9
[From Still's disease to chronic juvenile polyarthritis].[从斯蒂尔病到慢性青少年特发性关节炎]
An R Acad Nac Med (Madr). 1989;106(4):617-32.
10
[Acute pericarditis as the initial manifestation in Still's disease in an adult].[急性心包炎作为成人斯蒂尔病的首发表现]
Rev Esp Cardiol. 1991 Mar;44(3):210-2.

引用本文的文献

1
Wissler-Fanconi syndrome and related diagnoses: a case report.
Open Access Rheumatol. 2016 Jun 29;8:73-75. doi: 10.2147/OARRR.S105338. eCollection 2016.