Autoinflammatory syndromes in adults: report of 8 cases

Retrospective study documenting the clinical features, demographic and analytical parameters and therapeutic management in a cohort of adult patients with AIS.

Eight patients (50% male) were included, the majority of whom were Caucasian. The most frequent AIS was AOSD (37,5%). The mean age at symptom onset was 40 ± 23,1 years. The mean diagnostic delay was 5,7 ± 5 years. Fever, skin rash and myalgias were the most prevalent symptoms. The mean CRP value was 187,1 ± 110 mg/L and ESR rate of 95,2 ± 38 mm/h. 100% were treated with systemic corticosteroids, 50% with int i-IL-1. 3 patients required a switch from anakinra to canakinumab due to intolerance or lack of efficacy.

Given its increasing prevalence, this study emphasizes the importance of not underestimating the existence of AIS in adults, regardless of the level of health care. Early diagnosis and treatment with i-IL-1 are essential to control disease activity and prevent serious complications.