Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 505, Banpo-dong, Seocho-gu, Seoul, 137-040, Republic of Korea.
J Rheumatol. 2012 Jan;39(1):86-93. doi: 10.3899/jrheum.110639. Epub 2011 Dec 15.
To determine the characteristics of hemophagocytic syndrome (HPS) in adult Korean patients with systemic lupus erythematosus (SLE).
We reviewed the medical records of 1033 adult patients with SLE for a recent 14-year period and identified 15 patients who had developed HPS. Forty-two age- and sex-matched patients with SLE admitted for other manifestations were included as disease controls. Features of HPS in these patients were analyzed.
Reactive HPS occurred from some distinct causes during the course of SLE. HPS was associated with SLE in 11 patients (4 at onset of SLE and 7 at SLE flare), infection in 3 patients (2 bacterial infection; 1 viral infection), and drug use (azathioprine) in 1 patient. Common clinical features included fever (93.3%), hepatomegaly (60.0%), and splenomegaly (60.0%). Steroid pulse therapy (46.7%), immunosuppressants (46.7%), and intravenous immunoglobulin (46.7%) were frequently used for treatment of HPS. One patient (6.7%) died. Compared with SLE patients without HPS, those with HPS showed a higher SLEDAI score (p = 0.003) and lower levels of plasma leukocytes (p < 0.001), hemoglobin (p = 0.013), and platelets (p < 0.001) as well as a higher serum C-reactive protein level (p = 0.039) and a lower serum albumin level (p = 0.004).
HPS was observed in 1.5% of adult Korean patients with SLE. The occurrence of HPS was most frequently associated with the SLE disease activity. Profound pancytopenia, a high SLEDAI score, and notable changes in the level of acute-phase reactants can be the characteristics of SLE patients with HPS.
确定成人韩国系统性红斑狼疮（SLE）患者噬血细胞综合征（HPS）的特征。
我们回顾了最近 14 年期间 1033 例成人 SLE 患者的病历，发现 15 例患者发生了 HPS。纳入 42 例年龄和性别匹配的因其他表现入院的 SLE 患者作为疾病对照。分析这些患者的 HPS 特征。
反应性 HPS 在 SLE 病程中由一些明确的原因引起。HPS 与 SLE 相关（11 例患者，SLE 发病时 4 例，SLE 发作时 7 例）、感染（3 例患者，2 例细菌感染，1 例病毒感染）和药物使用（硫唑嘌呤）各 1 例。常见的临床特征包括发热（93.3%）、肝肿大（60.0%）和脾肿大（60.0%）。经常使用类固醇脉冲治疗（46.7%）、免疫抑制剂（46.7%）和静脉注射免疫球蛋白（46.7%）治疗 HPS。1 例患者（6.7%）死亡。与无 HPS 的 SLE 患者相比，HPS 患者的 SLEDAI 评分更高（p=0.003），血浆白细胞计数（p<0.001）、血红蛋白（p=0.013）和血小板计数（p<0.001）更低，血清 C 反应蛋白水平更高（p=0.039），血清白蛋白水平更低（p=0.004）。
在 1.5%的成人韩国 SLE 患者中观察到 HPS。HPS 的发生最常与 SLE 疾病活动相关。严重的全血细胞减少症、高 SLEDAI 评分和急性反应蛋白水平的显著变化可能是 HPS 患者的特征。
