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Evaluation of macrophage activation syndrome in hospitalised patients with Kikuchi-Fujimoto disease based on the 2016 EULAR/ACR/PRINTO classification criteria

Authors
 Sung Soo Ahn  ;  Byeori Lee  ;  Dam Kim  ;  Seung Min Jung  ;  Sang-Won Lee  ;  Min-Chan Park  ;  Yong-Beom Park  ;  Yong Gil Hwang  ;  Jason Jungsik Song 
Citation
 PLoS One, Vol.14(7) : e0219970, 2019 
Journal Title
 PLoS One 
Issue Date
2019
Abstract
BACKGROUND: To evaluate the impact of macrophage activation syndrome (MAS) on clinical features in patients with Kikuchi-Fujimoto disease (KFD) and to compare the features of MAS in KFD with those of adult-onset Still's disease (AOSD) and systemic lupus erythematosus (SLE). METHODS: The medical records of febrile patients hospitalised with KFD between November 2005 and April 2017 were reviewed. Patients fulfilling the 2016 classification criteria for MAS were classified as having MAS. Clinical and laboratory features of patients with KFD with and without MAS were evaluated. Poor hospitalisation outcomes were defined as intensive care unit admission or in-hospital mortality. The treatment outcomes of MAS in KFD, AOSD, and SLE were also compared. RESULTS: Among 78 patients hospitalised with KFD, 24 (30.8%) patients had MAS during admission. Patients with KFD and MAS more frequently required glucocorticoid treatment (66.7% vs 40.7%, p = 0.036) and had longer hospital stays than patients with KFD without MAS (12.5 vs 8.5 days, p<0.001). In addition, patients with MAS had worse hospitalisation outcomes than patients without MAS (29.2% vs. 0.0%, p<0.001). Among patients with MAS in KFD, AOSD, and SLE, the number of patients requiring glucocorticoid treatment after 3 months was significantly lower among patients with MAS and KFD (KFD 33.3%, AOSD 88.9%, SLE 100%, p<0.001). CONCLUSIONS: The presence of MAS in KFD was associated with adverse clinical outcomes including higher steroid usage and worse hospitalisation outcomes. However, compared to those with AOSD and SLE, patients with MAS and KFD were less likely to require long-term glucocorticoid treatment.
Files in This Item:
T201903286.pdf Download
DOI
10.1371/journal.pone.0219970
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Park, Min Chan(박민찬) ORCID logo https://orcid.org/0000-0003-1189-7637
Park, Yong Beom(박용범)
Song, Jungsik Jason(송정식) ORCID logo https://orcid.org/0000-0003-0662-7704
Ahn, Sung Soo(안성수) ORCID logo https://orcid.org/0000-0002-9002-9880
Lee, Sang Won(이상원) ORCID logo https://orcid.org/0000-0002-8038-3341
Jung, Seung Min (정승민) ORCID logo https://orcid.org/0000-0003-3465-2181
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/171328
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