• Kenji Saito, Tomoyuki Asano, Haruki Matsumoto, Yuya Fujita, Naoki Matsuoka, Hiroshi Ohkawara, Yuya Sumichika, Shuhei Yoshida, Jumpei Temmoku, Makiko Yashiro-Furuya, Shuzo Sato, Hiroshi Watanabe, and Kiyoshi Migita.
• Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan.
• Medicine (Baltimore). 2022 Dec 9; 101 (49): e32107e32107.

RationaleAdult-onset Still's disease (AOSD) is a rare inflammatory disease characterized by a classic triad of daily spike fever, arthritis, and a typical salmon-pink rash. The involvement of inflammatory cytokines by various factors such as infection, drug, or neoplasm causes refractory AOSD.Patient ConcernsWe report a 63-year-old man with a high fever, rash, hyperferritinemia, and M proteinemia. His serum levels of interleukin-6 and interleukin-18 were remarkably high at 192 and 114,250 pg/mL, respectively.DiagnosisAOSD complicated with monoclonal gammopathy of undetermined significance was diagnosed.InterventionsAfter steroid pulse therapy followed by oral prednisolone, cyclosporin, methotrexate, and colchicine, serum ferritin levels temporarily declined, but secondary cytomegalovirus infections exacerbated AOSD's activity.OutcomesFinally, after tocilizumab induction, AOSD activity was gradually suppressed over a long period.LessonsThe disease activity of AOSD is exacerbated by multiple factors, including comorbidities or infections. Clinicians need to consider that monoclonal gammopathy of undetermined significance complications might become AOSD refractory by an elevation of the inflammatory cytokines. Moreover, further prospective studies are required to confirm this result.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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