Validation of the Fautrel classification criteria for adult-onset Still’s disease

An atypical persistent eruption of adult‐onset Still's disease with neutrophilic urticarial dermatosis‐like dermal features: A case report and review of the literature

“…Serological abnormalities include mainly leukocytosis, elevated granulocyte counts, and abnormal liver function tests. These clinical and serological features are referred to as the standard Yamaguchi's criteria . With 5 or more Yamaguchi's criteria, including 2 or more major criteria, a sensitivity of 96.2%, and a specificity of 92.1% are reached.…”

“…In 1996, Masson et al compared criteria in AOSD; Yamaguchi's criteria were the most sensitive (93.5%), followed by Cush's (80.6%) and Calabro's (80.6%). Additionally, Fautrel et al proposed low glycosylated ferritin fraction as a specific marker of AOSD, with a sensitivity of 43.2% and a specificity of 92.9%. They compared Fautrel classification and Yamaguchi's criteria; the specificity of the Yamaguchi's criteria remained higher in this study.…”

“…Diagnosis of atypical persistent eruption of AOSD was made because more than five of Yamaguchi's criteria were fulfilled, and infectious and autoimmune diseases were ruled out. Additionally, high levels of serum ferritin (15,958 μg/L) with very low levels of glycosylated ferritin (<5%) were highly suggestive of this diagnosis . Oral corticosteroid (1 mg/kg) treatment was started.…”

Adult-Onset Still’s Disease

“…However, because of the substantial advances in AoSD diagnosis as well as differential diagnoses during the last two decades, these prevalence and incidence estimates lack robustness, and new studies are clearly needed to update these figures. Initially, the disease was characterized as affecting exclusively young adults (i.e., 16-35 years of age [1]); however, more recent series identified cases in adults older than 35 and even elderly people [5,[11][12][13][14]. The sex ratio is almost balanced, with only a slight female predominance [5,6,11,[13][14][15]].…”

“…The Fautrel criteria set has the advantage of including ferritin and GF levels as diagnostic biomarkers and does not require exclusion criteria [13]. In a recent validation study, both sets showed high sensitivity and specificity [14].…”