Dokkyo Medical Journal
Online ISSN : 2436-522X
Print ISSN : 2436-5211

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Clinical Abdominal Kikuchi-Fujimoto Disease Resembling Macrophage Activation Syndrome Associated with Systemic Juvenile Idiopathic Arthritis: A Case Report
Yuji FujitaYusuke AndoTamae KatoShigeko KuwashimaShigemi Yoshihara
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ジャーナル オープンアクセス 早期公開

論文ID: 2022-038

この記事には本公開記事があります。
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A 13-year-old girl presented with fever for the past ten days without any other symptoms. Laboratory findings revealed leukopenia, low platelet count, and high lactate dehydrogenase and ferritin levels. Abdominal ultrasonography revealed mild splenomegaly and lymphadenopathy in proximity to the head of the pancreas. The patient's condition suggested macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. However, her serum interleukin-18 level was 1743 pg/mL; therefore, urgent systemic corticosteroid treatment was deemed unnecessary as macrophage activation syndrome associated with systemic juvenile idiopathic arthritis was ruled out. The patient was afebrile the following day and showed improved laboratory and ultrasonographic findings without treatment. Clinical Kikuchi-Fujimoto disease was diagnosed based on transient lymphadenopathy, laboratory findings, and a self-limited clinical course. Although this patient met the early diagnostic criteria of macrophage activation syndrome with suspected systemic juvenile idiopathic arthritis, serum interleukin-18 is useful for ruling it out. Pediatricians should keep in mind that Kikuchi-Fujimoto disease may cause abdominal lymphadenopathy.

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