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 a 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 2 clinical subsets of AOSD and investigated the clinically significant characteristics of the 2 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 of 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 interleukin-18 (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-cyclic citrullinated peptide antibodies and 1 patient with the non-RA subtype had rheumatoid factor. These findings distinguish these patients from patients with true RA. Conclusion. There were 2 subsets of patients with AOSD in the examined population. Patients with high levels of IL-18 or ferritin presented with severe systemic inflammatory disorders (non-RA subtype) and patients with low levels of IL-18 or ferritin developed severe arthritis (RA subtype).

Cytokine profile in adult-onset Still's disease: Comparison with systemic juvenile idiopathic arthritis

“…Patients with severe systemic inflammatory disorders (non-RA subtype) presented with high IL-18 levels, whereas those that developed severe arthritis (RA subtype) presented with low IL-18 levels [54]. The present study findings were consistent with those earlier findings and indicate that s-JIA and AOSD might be pathogenically identical.…”

“…Interestingly, a recent study reported the existence of two subgroups of patients with AOSD that were based on serum IL-18 levels [54]. Patients with severe systemic inflammatory disorders (non-RA subtype) presented with high IL-18 levels, whereas those that developed severe arthritis (RA subtype) presented with low IL-18 levels [54].…”

Adult onset Still’s disease presenting as fever of unknown origin: a case report with review of literature

“…IL-18 and serum ferritin levels correlate with disease activity in AOSD. 15,17 Rheumatoid factor and ANA are found negative in 93% and 92% of cases respectively.…”

Clinical Predictors in Chronic Articular Adult‐Onset Still's Disease: Comment on the Article by Ichida et al

“…We read with great interest the article by Ichida et al published recently in Arthritis Care & Research regarding the clinical manifestations of a large cohort of patients with adult-onset Still's disease (AOSD) (1). We fully agree with the authors, and we would like to make a brief comment on both the possibility of erosive arthritis and the need for reliable clinical predictors of outcome to treat arthritis aggressively as soon as possible.…”