Department of General Internal Medicine, NHO National Nagasaki Medical Center, 2-1001-1 Kubara, Omura 856-8652, Japan.
Mod Rheumatol. 2012 Jun;22(3):483-7. doi: 10.1007/s10165-011-0543-3. Epub 2011 Oct 22.
This is the first report on the efficacy of leukocytapheresis (LCAP) in a patient with refractory systemic-type adult-onset Still's disease (AOSD). A 17-year-old Japanese woman with AOSD who had been treated with prednisolone and cyclosporine A presented with relapse of typical systemic AOSD, including high fever, rash, and liver dysfunction. Steroid pulse therapy (methylprednisolone 500 mg/day) was performed, which failed to stabilize the disease. Therefore, LCAP (twice/week for a total of five courses) was introduced in combination with high-dose steroids plus cyclosporin A. Elevated levels of serum ferritin and transaminases and neutrophil CD64 expression normalized after the patient's disease was successfully controlled by the induction of LCAP. In this case, elevated levels of interleukin (IL)-1β and IL-18 were normalized after LCAP induction, suggesting that LCAP treatment modulates the deregulated cytokine-mediated inflammatory responses observed in AOSD. Our clinical observations suggest that LCAP may be beneficial for flare-up of systemic manifestations of AOSD refractory to conventional treatment, including high-dose steroids and immunosuppressants.
这是首例关于白细胞吸附疗法（LCAP）治疗难治性全身型成人Still 病（AOSD）疗效的报告。一名 17 岁的日本女性患有 AOSD，曾接受泼尼松龙和环孢素 A 治疗，出现典型的全身型 AOSD 复发，包括高热、皮疹和肝功能障碍。给予了类固醇脉冲疗法（甲泼尼龙 500mg/天），但未能稳定病情。因此，联合大剂量类固醇加环孢素 A 引入了 LCAP（每周两次，共 5 个疗程）。在成功诱导 LCAP 控制疾病后，患者血清铁蛋白和转氨酶水平升高以及中性粒细胞 CD64 表达恢复正常。在该病例中，LCAP 诱导后白细胞介素（IL）-1β和 IL-18 水平恢复正常，表明 LCAP 治疗可调节 AOSD 中观察到的失调细胞因子介导的炎症反应。我们的临床观察表明，LCAP 可能对包括大剂量类固醇和免疫抑制剂在内的常规治疗难治性的 AOSD 全身表现的发作有益。
