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成人斯蒂尔病:诊断问题

[Still's disease in adults: diagnostic problems].

作者信息

Foresti V, Casati O, Zubani R, Parisio E, Confalonieri F

机构信息

Divisione medica III, Ospedale Fatebenefratelli e oftalmico, Presidio multizonale ospedaliero dell'E.R.S.Z., Milano.

出版信息

Recenti Prog Med. 1990 Jul-Aug;81(7-8):479-81.

PMID:2247694
Abstract

Still's disease is a seronegative arthritis of children which, in a limited number of cases, can affect adults. The diagnosis of adult-onset Still's disease is characterized by high fever, arthritis and negative serologic tests for rheumatoid factor and antinuclear antibodies and by at least two minor symptoms (leukocytosis, evanescent rash, serositis, hepato- or splenomegaly, and lympho-adenopathy). Since many diseases present analogous manifestations and the adult-onset Still's disease is generally diagnosed by exclusion, we report two patients, aged 26 and 39, with Still's disease, the former with a classic clinical feature, the latter with a clinical feature characterized by severe hepatic abnormalities. The determination of histocompatibility antigens can be useful because some of them (HLA-DR4 in case 1 and HLA-DRw6 in case 2) are frequently associated with the adult-onset Still's disease. The role of anti-inflammatory therapy (acetylsalicylic acid, indomethacin, steroids) must be emphasized, whose efficacy can constitute the pathognomonic element on which the diagnosis of adult-onset Still's disease can be based in a proper clinical pattern.

摘要

斯蒂尔病是一种儿童血清阴性关节炎,在少数情况下也可影响成人。成人斯蒂尔病的诊断特征为高热、关节炎、类风湿因子和抗核抗体血清学检测阴性,以及至少两种次要症状(白细胞增多、一过性皮疹、浆膜炎、肝肿大或脾肿大、淋巴结病)。由于许多疾病有类似表现,且成人斯蒂尔病通常通过排除法诊断,我们报告了两名年龄分别为26岁和39岁的斯蒂尔病患者,前者具有典型临床特征,后者以严重肝脏异常为临床特征。组织相容性抗原的测定可能有用,因为其中一些抗原(病例1中的HLA - DR4和病例2中的HLA - DRw6)常与成人斯蒂尔病相关。必须强调抗炎治疗(乙酰水杨酸、吲哚美辛、类固醇)的作用,其疗效可构成在适当临床模式下诊断成人斯蒂尔病所依据的特征性要素。

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