Division of Internal Medicine, Faculty of Health Sciences, School of Clinical Medicine, University of Witwatersrand, Johannesburg, South Africa.
Department of Rheumatology, Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.
Am J Case Rep. 2023 Jun 23;24:e939520. doi: 10.12659/AJCR.939520.
BACKGROUND Still's disease is a rare multisystemic autoinflammatory disorder. The diagnosis of adult-onset Still's disease (AoSD) can be challenging due to the rarity and overlapping features with many other systemic disorders. Complications of the illness can involve many systems in the human body. One of the least documented hematological complications of AoSD is thromboembolic phenomena. CASE REPORT This text outlines the presentation of a 43-year-old woman with a known diagnosis of AoSD, whose disease-modifying anti-rheumatic drugs (DMARDs) had been tapered and stopped due to remission. She presented with respiratory symptoms and features of an AoSD flare. Lack of complete improvement on antibiotic therapy and reinitiating of DMARDs prompted seeking an alternative/concurrent diagnosis. The work-up yielded a pulmonary embolism (PE) on the background of having no other risk factors for thrombosis. CONCLUSIONS In the reviewed literature, there is a close association between hyperferritinemia and AoSD complicated with venous thromboemboli (VTEs). A rigorous search for alternative diagnoses as well as other potential uncommon complications of AoSD is needed when working-up patients with AoSD, especially those that are not getting better on therapy. Given the rarity of AoSD, meticulous data collection may be useful in understanding the pathophysiology and features of presentation of the illness, including complications such as VTEs.
斯蒂尔病是一种罕见的多系统自身炎症性疾病。由于成人斯蒂尔病(AoSD)的罕见性和与许多其他系统性疾病的重叠特征,其诊断具有一定挑战性。疾病的并发症可能涉及人体的许多系统。AoSD 最少记录的血液学并发症之一是血栓栓塞现象。
本文概述了一名 43 岁女性的表现,她被诊断为 AoSD,由于缓解,她的疾病修饰抗风湿药物(DMARDs)已逐渐减少并停止。她出现了呼吸道症状和 AoSD 发作的特征。抗生素治疗效果不佳,重新开始 DMARDs 治疗促使寻找替代/并发诊断。在没有其他血栓形成危险因素的情况下,检查结果显示存在肺栓塞(PE)。
在回顾的文献中,高血清铁蛋白与 AoSD 并发静脉血栓栓塞(VTE)之间存在密切关联。在对 AoSD 患者进行检查时,需要仔细寻找其他替代诊断和 AoSD 的其他潜在罕见并发症,特别是那些在治疗后没有好转的患者。鉴于 AoSD 的罕见性,仔细收集数据可能有助于了解疾病的病理生理学和表现特征,包括 VTE 等并发症。