Adult-onset Still's disease
Adult-onset Still's disease
复制标题
DOI：
10.1016/j.berh.2004.05.004
复制
发表时间：
2004-10-01
影响因子：
5.2
通讯作者：
Petrovicz, E
中科院分区：
文献类型：
--
作者：
Kádár, J;Petrovicz, E
成人发病的斯蒂尔氏病（AOSD）是一种病因和发病机制尚不清楚的全身性炎症性疾病。AOSD是一种罕见的疾病，通常表现为高烧并伴有全身表现。该疾病是一种异质性的病理实体，具有一系列的病因、表现和预后。AOSD没有单一的诊断测试；相反，诊断是基于临床标准，如关节痛、发热、皮疹、淋巴结病和肝脾肿大。测定降钙素原水平和对经验性皮质类固醇治疗的生物学反应通常有助于诊断，而免疫血清学作为一种“筛选”试验，在大多数情况下不能提供有意义的信息。治疗包括抗炎药物。非甾体类抗炎药物疗效有限，通常需要皮质类固醇治疗和改善疾病的抗风湿药物。新的治疗方法，如抗肿瘤坏死因子阻断和干细胞移植，是有希望的。在本章中，我们介绍了1997年至2003年在我院诊断为AOSD的IS患者的临床和实验室参数，并回顾了文献。
Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis. AOSD is a rare condition, usually presenting with high fever accompanied by systemic manifestations. The disease is a heterogeneous pathological entity with a range of etiologies, manifestations and prognosis.There is no single diagnostic test for AOSD; rather, the diagnosis is based upon clinical criteria such as arthralgia, fever, skin rash, lymphadenopathy, and hepatosplenomegaly. Determination of the procalcitonin level and the biological response to empirical corticosteroid therapy generally helps the diagnosis, while immune-serology, as a 'screening' test, will not add meaningful information in most cases. Treatment consists of anti-inflammatory medications. Non-steroid anti-inflammatory drugs have limited efficacy, corticosteroid therapy and disease-modifying antirheumatic drugs are usually required. Novel therapeutic approaches, such as anti-tumor necrosis factor blockade and stem cell transplantation, are promising. In this chapter we present clinical and laboratory parameters of IS patients diagnosed with AOSD at our institution between 1997 and 2003, and review the literature.