Department of Internal Medicine, Reinier de Graaf Group of Hospitals, PO Box 5011, 2600 GA Delft, The Netherlands.
Rheumatol Int. 2012 Aug;32(8):2487-90. doi: 10.1007/s00296-011-1991-y. Epub 2011 Jul 21.
Adult-onset Still's disease (AOSD) is known as a systemic inflammatory disease of unknown etiology and pathogenesis, characterized by fever, skin eruptions, systemic organ involvement, and arthralgias. AOSD is difficult to diagnose because of its heterogeneous clinical manifestations and prevalence (although more prevalent in the young, onset of AOSD after the age of 60 has also been described), and absence of pathognomonic clinical features. The disease also lacks a specific diagnostic test. To date, association studies between AOSD and HLA loci have failed to indentify a genetic predisposition. The recent publication of entirely different PET-CT manifestations found in three patients who were supposed to have the same disease (AOSD), as well as the surprisingly different PET-CT images of our AOSD patient (accumulation in the carotids and large vessels of the legs), raises our suspicion that AOSD is actually not one entity but a constellation of disorders whose varying underlying pathologies are now being revealed by new imaging techniques.
成人Still 病(AOSD)是一种病因和发病机制不明的系统性炎症性疾病,其特征为发热、皮疹、全身器官受累和关节痛。由于其临床表现和流行情况(尽管在年轻人中更为常见,但也有 60 岁以后发病的 AOSD 病例)存在异质性,且缺乏特征性的临床特征,因此该病的诊断较为困难。此外,该病也缺乏特定的诊断试验。迄今为止,AOSD 与 HLA 基因座之间的关联研究并未确定遗传易感性。最近发表的三篇病例报告中,三名被认为患有同种疾病(AOSD)的患者的 PET-CT 表现完全不同,而且我们的 AOSD 患者的 PET-CT 图像也出乎意料地不同(颈动脉和腿部大血管的聚集),这使我们怀疑 AOSD 实际上并不是一种单一的疾病实体,而是一种由不同潜在病理引起的病症组合,而新的成像技术正在揭示这些不同的潜在病理。