Abstract
BackgroundTo 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).MethodsThe 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.ResultsAmong 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).ConclusionsThe 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.
Highlights
Kikuchi-Fujimoto disease (KFD), known as histiocytic necrotising lymphadenitis, is a rare idiopathic inflammatory disease first described in 1972 by Kikuchi and Fujimoto et al [1, 2]
Among patients with macrophage activation syndrome (MAS) in KFD, adult-onset Still’s disease (AOSD), and systemic lupus erythematosus (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
The presence of MAS in KFD was associated with adverse clinical outcomes including higher steroid usage and worse hospitalisation outcomes
Summary
Kikuchi-Fujimoto disease (KFD), known as histiocytic necrotising lymphadenitis, is a rare idiopathic inflammatory disease first described in 1972 by Kikuchi and Fujimoto et al [1, 2]. Some patients manifest non-specific systemic symptoms including night sweats, weight loss, nausea, vomiting, hepatosplenomegaly, and headache [6, 7]. The clinical characteristics of KFD can resemble those of malignant lymphoma, metastatic carcinoma, infectious lymphadenitis, systemic autoimmune diseases, and Mycobacterium tuberculosis infections [8,9,10]. 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).
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