title: Clinical manifestations of Adult-onset Still's disease presenting with erosive arthritis: Association with low levels of ferritin and Interleukin-18.
abstract: Objective: Adult-onset Still's disease (AOSD) is a clinical entity with heterogeneous etiology. We have encountered patients with AOSD who had severe polyarthritis and who fulfilled the classification criteria for rheumatoid arthritis (RA); however, most patients with AOSD typically exhibit mild arthritis. In this study, we proposed two clinical subsets of AOSD and investigated the clinically significant characteristics of the two subtypes. Methods: We retrospectively analyzed 71 consecutive patients with AOSD. We reviewed the medical records of all patients who were followed up for more than 2 years. We classified all the patients with AOSD into the following 2 subsets: an RA subtype for patients who met the criteria for RA according to the American College of Rheumatology and a non-RA subtype for patients who did not meet the criteria for RA. Results: Our results indicated that the non-RA subtype was accompanied by severe inflammatory complications, including pleuritis and hemophagocytic syndrome. In addition, the serum ferritin and serum IL-18 levels were significantly higher in patients with the non-RA subtype than in those with the RA subtype. Interestingly, only 1 patient with the RA subtype had anti-CCP antibodies, and 1 non-RA subtype patient had rheumatoid factor. These findings distinguish these patients from patients with true RA. Conclusions: There were two subsets of patients with AOSD in the examined population. Patients with high levels of IL-18 or ferritin presented with severe systemic inflammatory disorders (the non-RA subtype), and patients with low levels of IL-18 or ferritin developed severe arthritis (RA subtype).
authors.affiliation: Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Hisae
authors.initials: H
authors.lastname: Ichida
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Kawaguchi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Sugiura
authors.forename: Kae
authors.initials: K
authors.lastname: Takagi
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Katsumata
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Yuko
authors.initials: Y
authors.lastname: Ota
authors.forename: Sayuri
authors.initials: S
authors.lastname: Kataoka
authors.forename: Hidenaga
authors.initials: H
authors.lastname: Kawasumi
authors.forename: Hisashi
authors.initials: H
authors.lastname: Yamanaka
mesh_terms: D000293:Adolescent; D000328:Adult; D001172:Arthritis, Rheumatoid / Q000097:blood* / Q000150:complications / Q000473:pathology; D005260:Female; D005293:Ferritins / Q000097:blood*; D006801:Humans; D007249:Inflammation; D020382:Interleukin-18 / Q000097:blood*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016706:Still's Disease, Adult-Onset / Q000097:blood* / Q000145:classification / Q000150:complications; D055815:Young Adult
chemical_list: D020382:Interleukin-18; D005293:Ferritins