Department of Medicine, Jinnah Medical College Hospital, Karachi, Pakistan.
Int J Rheum Dis. 2012 Oct;15(5):e96-100. doi: 10.1111/j.1756-185X.2012.01812.x. Epub 2012 Sep 17.
Adult-onset Still's disease (AOSD) is a rare disease. Very few cases have been reported from the South-Asian region so the aim of this study is to assess the clinical and laboratory aspects of 15 patients with AOSD in a tertiary referral hospital in Karachi.
Retrospective data was collected from all patients diagnosed using Yamaguchi criteria for AOSD between January 2004 and December 2010 at Jinnah Medical College Hospital, Karachi.
Data of 15 patients with AOSD were analyzed. Their ages ranged from 17 to 55 years, the male-to-female ratio being 6:1. The most common clinical features were fever and articular symptoms (100%), sore throat (60%), rash (53.3%), weight loss (93.3%), lymphadenopathy (40%) and elevated erythrocyte sedimentation rate (86.7%). All patients had leukocytosis with counts>20,000/mm 3 were seen in 40%. Elevated liver enzymes were present in 80% of the case series and hyperferritinemia in 100% with a mean of 3,962 ng/mL (range 555-13,865). Ambiguity in presentation and lack of serologic markers make diagnosis of AOSD difficult as 40% of patients were receiving empirical anti-tuberculous therapy prior to final diagnosis.
It is necessary for physicians to have a high index of suspicion for AOSD in patients with high-grade fever, arthralgia and leukocytosis.
成人Still 病（AOSD）是一种罕见疾病。南亚地区报道的病例非常少，因此本研究旨在评估卡拉奇一家三级转诊医院的 15 例 AOSD 患者的临床和实验室特征。
本研究回顾性收集了 2004 年 1 月至 2010 年 12 月期间在卡拉奇真纳医学院医院根据 Yamaguchi 标准诊断为 AOSD 的所有患者的数据。
分析了 15 例 AOSD 患者的数据。他们的年龄在 17 至 55 岁之间，男女比例为 6:1。最常见的临床特征是发热和关节症状（100%）、咽痛（60%）、皮疹（53.3%）、体重减轻（93.3%）、淋巴结病（40%）和红细胞沉降率升高（86.7%）。所有患者均有白细胞计数升高，计数>20,000/mm 3 的占 40%。80%的病例系列存在肝酶升高，100%的病例存在高血清铁蛋白血症，平均值为 3,962ng/mL（范围 555-13,865）。由于表现不明确且缺乏血清学标志物，使得 AOSD 的诊断变得困难，40%的患者在最终诊断前接受了经验性抗结核治疗。
对于高热、关节炎和白细胞增多的患者，医生有必要高度怀疑 AOSD。
