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Macrophage activation syndrome in systemic lupus erythematosus: a multicenter, case-control study in China

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Abstract

The objective of this study was to describe the clinical and laboratory characteristics, precipitating factors, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014. A total of 32 patients with SLE-associated MAS were enrolled. Sixty-four age- and sex-matched SLE patients diagnosed in the same period without MAS episodes were selected as controls. The most frequent clinical feature was fever, followed by splenomegaly. Hyperferritinemia, hypoalbuminemia, and hyper-lactate dehydrogenase (LDH)-nemia were among the most common laboratory abnormalities. Compared with pre-MAS visit, patients at the onset of MAS had greater frequencies of renal involvement, liver dysfunction, and cytopenia. Receiver operating characteristic (ROC) analysis identified optimal cutoff values of ferritin (>662.5 ng/mL) and LDH (>359 U/mL) to predict the occurrence of MAS in SLE. SLE flare and infection were the common triggers of MAS in SLE. Abortion and parturition were recorded as well. The overall mortality rate was 12.5%. All patients received corticosteroids. Cyclosporine A, cyclophosphamide, and etoposide were the three most commonly used immunosuppressants. Rituximab was given to one patient. Intravenous immunoglobulin (IVIG) was added for 46.9% patients. MAS is a potentially fatal complication of SLE. Its occurrence is most frequently associated with active SLE disease or infection. The presentation of unexplained fever, cytopenia, or liver dysfunction, with high levels of ferritin and LDH, in patients with SLE should raise the suspicion of MAS. Corticosteroids with immunosuppressants and IVIG may be an appropriate treatment.

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Acknowledgements

This work was supported by the National Natural Science Foundation of China (No. 31300721).

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  1. Ai-Chun Liu and Yue Yang contributed equally to the manuscript.

Authors and Affiliations

  1. Department of Rheumatology and Immunology, Peking University People’s Hospital and Key Laboratory of Rheumatism Mechanism and Immune Diagnosis (BZ0135) and Peking-Tsinghua Center for Life Science, Beijing, China

    Ai-Chun Liu, Yue Yang, Yuan Jia & Zhan-Guo Li

  2. Department of Rheumatology, Peking Union Medical College Hospital and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China

    Meng-Tao Li, Yan Zhao & Xiao-Feng Zeng

  3. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China

    Sheng Chen & Shuang Ye

  4. Department of Hematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China

    Xiang-Zong Zeng & Zhao Wang

  5. Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China

    Jin-Xia Zhao & Xiang-Yuan Liu

  6. Department of Rheumatology, General Hospital of the People’s Liberation Army, Beijing, China

    Jian Zhu

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  1. Ai-Chun Liu
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  2. Yue Yang
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Correspondence to Meng-Tao Li or Yuan Jia.

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This study was approved by the Institutional Research Ethics Committees and carried out in compliance with the Helsinki Declaration. Both of the patients groups gave written informed consent.

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Liu, AC., Yang, Y., Li, MT. et al. Macrophage activation syndrome in systemic lupus erythematosus: a multicenter, case-control study in China. Clin Rheumatol 37, 93–100 (2018). https://doi.org/10.1007/s10067-017-3625-6

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