Nagalli Shivaraj, Sharma Ashish, Shankar Kikkeri Nidhi, Sherif Nasef
Internal Medicine, Brookwood Baptist Health, Yuma, USA.
Internal Medicine, Yuma Regional Medical Center, Yuma, USA.
Cureus. 2020 Aug 28;12(8):e10098. doi: 10.7759/cureus.10098.
Adult-onset Still's disease (AOSD) is a rare autoimmune disease of unknown etiology with systemic inflammatory manifestations consisting of a triad of fevers, evanescent skin rash, and arthritis. Although steroids are the first line of therapy, about 20%-30% of patients are refractory, intolerant, and or relapse during tapering or upon discontinuation of steroids. There are no clinical guidelines in treating such patients and treatment in these patients is challenging. Previously used biological agents have limited efficacy and hence there is a need for new therapies. Tocilizumab (TCZ), an interleukin (IL)-6 receptor antibody has been used with a clinical benefit and has shown to decrease the dose of steroids in patients with adult-onset still disease. The aim of this case report is to highlight the use of tocilizumab in relapsing and steroid intolerant cases of AOSD. The use of this drug in patients with AOSD is currently off-label. Randomized control studies can provide additional information that offers better visibility in treating AOSD patients who are steroid-resistant or intolerant. The rarity of disease possesses additional challenges in conducting these studies.
成人斯蒂尔病(AOSD)是一种病因不明的罕见自身免疫性疾病,具有全身性炎症表现,包括发热、一过性皮疹和关节炎三联征。尽管类固醇是一线治疗药物,但约20%-30%的患者难治、不耐受,且在类固醇减量或停药期间复发。治疗这类患者尚无临床指南,对这些患者的治疗具有挑战性。先前使用的生物制剂疗效有限,因此需要新的治疗方法。托珠单抗(TCZ),一种白细胞介素(IL)-6受体抗体,已被用于临床并显示出益处,且已证明可降低成人斯蒂尔病患者的类固醇剂量。本病例报告的目的是强调托珠单抗在复发性和类固醇不耐受的AOSD病例中的应用。目前该药物在AOSD患者中的使用属于超说明书用药。随机对照研究可以提供更多信息,有助于更好地治疗对类固醇耐药或不耐受的AOSD患者。疾病的罕见性给开展这些研究带来了额外的挑战。