Ji-Min Kim1, Seung-Ki Kwok, Ji Hyeon Ju, Ho-Youn Kim, Sung-Hwan Park. 1. 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.
Abstract
OBJECTIVE: To determine the characteristics of hemophagocytic syndrome (HPS) in adult Korean patients with systemic lupus erythematosus (SLE). METHODS: 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. RESULTS: 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). CONCLUSION: 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.
OBJECTIVE: To determine the characteristics of hemophagocytic syndrome (HPS) in adult Korean patients with systemic lupus erythematosus (SLE). METHODS: 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. RESULTS: 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 SLEpatients 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). CONCLUSION: 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 SLEpatients with HPS.