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[成人斯蒂尔病——一条通过不明原因发热和积液进行诊断的艰难之路]

[Adult-onset Stills disease - a difficult path to diagnosis through fever and effusions of unknown origin].

作者信息

Malegová Jana, Koten Lukáš, Horák Pavel

出版信息

Vnitr Lek. 2014 May-Jun;60(5-6):520-6.

Abstract

Fever of unknown origin, pleural and pericardial effusions can be caused by a variety of independent agents. On the other hand, we can identify a common causative condition in other cases. Infectious diseases, malignancies and autoimmune diseases are the most common etiological factors. Considering the pleural and pericardial effusion, we also have to think of cardiovascular and pulmonary diseases. The basis of every diagnostic process is thorough medical history and detailed clinical examination followed by laboratory and imaging methods. In spite of that, the right diagnosis sometimes stays long time hidden. One of such conditions is Adult-onset Stills disease (AOSD). It is a rare inflammatory, potentially life-threatening disease with unclear pathogenesis and heterogeneous symptoms. It has some features similar to systemic form of juvenile idiopathic arthritis. Diagnosis is established so called per exclusionem by fulfilling a set of clinical criteria and ruling out other diseases with similar symptomatology. In our article, we present an example of such an arduous diagnostic journey to final diagnosis.

摘要

不明原因发热、胸腔和心包积液可由多种独立因素引起。另一方面,在其他病例中我们可以识别出一种常见的致病情况。传染病、恶性肿瘤和自身免疫性疾病是最常见的病因。考虑到胸腔和心包积液,我们还必须想到心血管和肺部疾病。每个诊断过程的基础都是详细的病史、细致的临床检查,随后进行实验室和影像学检查。尽管如此,正确的诊断有时仍会长期隐匿。成人斯蒂尔病(AOSD)就是其中之一。它是一种罕见的炎症性疾病,可能危及生命,发病机制不明,症状多样。它具有一些与幼年特发性关节炎全身型相似的特征。通过满足一系列临床标准并排除其他具有相似症状的疾病,即所谓的排除法来确立诊断。在我们的文章中,我们展示了这样一个艰难的诊断过程直至最终确诊的实例。

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