Tohoku J. Exp. Med., 2021 November, 255(4)

Adult-Onset Still’s Disease Complicated by Immunoglobulin A Vasculitis and anti-CCP Antibody-Positive Arthritis

Yuya Fujita,1 Shuzo Sato,1 Haruki Matsumoto,1 Jumpei Temmoku,1 Makiko Yashiro-Furuya,1 Naoki Matsuoka,1 Tomoyuki Asano,1 Kohei Yokose,1 Shuhei Yoshida,1 Mikio Ohtsuka,2 Hiroshi Watanabe1 and Kiyoshi Migita1

1Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan
2Department of Dermatology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan

A 38-year-old male was admitted to our hospital for arthralgia, fever, skin rash, and purpura. He was diagnosed as having adult-onset Still’s disease (AOSD) based on Yamaguchi’s criteria. Skin biopsy revealed immunoglobulin A (IgA) vasculitis. He was also found to have anti-cyclic citrullinated peptide (CCP) antibody-positive inflammatory arthritis on a shoulder joint, however he did not fulfill classification criteria for rheumatoid arthritis. Elevated serum cytokine such as serum IL-18 supported the diagnosis of AOSD. His symptoms improved with 40 mg of prednisolone plus cyclosporin A (200 mg/day). Two years after hospitalization, AOSD was relapsed with pleurisy and hyperferritinemia. Finally, he was diagnosed with multicyclic systemic type of AOSD complicated by IgA vasculitis and seropositivity of anti-CCP antibody. Clinicians need to consider the complication of multiple rheumatic diseases, even if the disease-specific autoantibody is positive.

Keywords —— adult-onset Still’s disease; anti-CCP antibody; IgA vasculitis; interleukin-18

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Tohoku J. Exp. Med 2021, 255, 291-296.

Correspondence: Yuya Fujita, M.D., Department of Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, Fukushima 960-1295, Japan.

e-mail: fujita31@fmu.ac.jp