The Tohoku Journal of Experimental Medicine
Online ISSN : 1349-3329
Print ISSN : 0040-8727
ISSN-L : 0040-8727
Case
Adult-Onset Still’s Disease Complicated by Immunoglobulin A Vasculitis and anti-CCP Antibody-Positive Arthritis
Yuya FujitaShuzo SatoHaruki MatsumotoJumpei TemmokuMakiko Yashiro-FuruyaNaoki MatsuokaTomoyuki AsanoKohei YokoseShuhei YoshidaMikio OhtsukaHiroshi WatanabeKiyoshi Migita
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2021 Volume 255 Issue 4 Pages 297-301

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Abstract

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.

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