PMID- 28409239 OWN - NLM STAT- MEDLINE DCOM- 20180803 LR - 20220801 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 1 DP - 2018 Jan TI - Macrophage activation syndrome in systemic lupus erythematosus: a multicenter, case-control study in China. PG - 93-100 LID - 10.1007/s10067-017-3625-6 [doi] AB - 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. FAU - Liu, Ai-Chun AU - Liu AC AD - 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. FAU - Yang, Yue AU - Yang Y AD - 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. FAU - Li, Meng-Tao AU - Li MT AD - 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. mengtao.li@cstar.org.cn. FAU - Jia, Yuan AU - Jia Y AD - 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. jiayuan1023@sina.com. FAU - Chen, Sheng AU - Chen S AD - Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Ye, Shuang AU - Ye S AD - Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Zeng, Xiang-Zong AU - Zeng XZ AD - Department of Hematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China. FAU - Wang, Zhao AU - Wang Z AD - Department of Hematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China. FAU - Zhao, Jin-Xia AU - Zhao JX AD - Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China. FAU - Liu, Xiang-Yuan AU - Liu XY AD - Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China. FAU - Zhu, Jian AU - Zhu J AD - Department of Rheumatology, General Hospital of the People's Liberation Army, Beijing, China. FAU - Zhao, Yan AU - Zhao Y AD - 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. FAU - Zeng, Xiao-Feng AU - Zeng XF AD - 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. FAU - Li, Zhan-Guo AU - Li ZG AD - 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. LA - eng GR - Nos 31300721/National Natural Science Foundation of China/ PT - Journal Article PT - Multicenter Study DEP - 20170413 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Adolescent MH - Adult MH - Case-Control Studies MH - China MH - Cyclophosphamide/therapeutic use MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/*complications/drug therapy MH - Macrophage Activation Syndrome/*complications/drug therapy MH - Male MH - Middle Aged MH - Retrospective Studies MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - Ferritins OT - Lupus erythematosus, systemic OT - Macrophage activation syndrome EDAT- 2017/04/15 06:00 MHDA- 2018/08/04 06:00 CRDT- 2017/04/15 06:00 PHST- 2017/03/29 00:00 [received] PHST- 2017/04/03 00:00 [accepted] PHST- 2017/04/15 06:00 [pubmed] PHST- 2018/08/04 06:00 [medline] PHST- 2017/04/15 06:00 [entrez] AID - 10.1007/s10067-017-3625-6 [pii] AID - 10.1007/s10067-017-3625-6 [doi] PST - ppublish SO - Clin Rheumatol. 2018 Jan;37(1):93-100. doi: 10.1007/s10067-017-3625-6. Epub 2017 Apr 13.