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

• Published in: PloS one 2019-07, Vol.14 (7), p.e0219970-e0219970
• Main Authors: Ahn, Sung Soo, Lee, Byeori, Kim, Dam, Jung, Seung Min, Lee, Sang-Won, Park, Min-Chan, Park, Yong-Beom, Hwang, Yong Gil, Song, Jason Jungsik
• Format: Article
• Language: English
• Subjects: Activation analysis; Adult; Arthritis; Autoimmune diseases; Biology and Life Sciences; Cell activation; Chronic conditions; Classification; Collaboration; Comorbidity; Complications and side effects; Cytokines; Development and progression; Female; Fever; Glucocorticoids; Glucocorticoids - therapeutic use; Histiocytic Necrotizing Lymphadenitis - complications; Histiocytic Necrotizing Lymphadenitis - diagnosis; Histiocytic Necrotizing Lymphadenitis - epidemiology; Histiocytic Necrotizing Lymphadenitis - therapy; Hospital Mortality; Hospitalization; Humans; Immunologic diseases; Immunology; Immunosuppressive Agents - therapeutic use; Intensive care; Internal medicine; Length of Stay; Lupus; Lupus erythematosus; Lymphadenitis; Lymphatic diseases; Macrophage Activation Syndrome - complications; Macrophage Activation Syndrome - diagnosis; Macrophage Activation Syndrome - epidemiology; Macrophage Activation Syndrome - therapy; Macrophages; Male; Medical records; Medicine; Medicine and Health Sciences; Mortality; Patients; Physiological aspects; Prognosis; Retrospective Studies; Rheumatism; Rheumatology; Risk factors; Steroids; Still's Disease, Adult-Onset; Systemic lupus erythematosus; Viral infections; Young Adult
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0219970

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). 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. 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