Reactive Hemophagocytic Syndrome in Adult Korean Patients with Systemic Lupus Erythematosus: A Case-Control Study and Literature Review

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.