Abstract

Background: Aim of this study was to search for any difference in the outcome of patients with adult onset Still's disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment.Patients and Methods: One hundred and forty-one AOSD patients treated with ANK have been retrospectively assessed. Statistically significant differences (p < 0.05) were analyzed in the frequency of ANK effectiveness, primary or secondary inefficacy to ANK and rate of resolution of clinical and laboratory AOSD manifestations after 3, 6, and 12 months since ANK treatment according with different lines of treatment and different times between AOSD onset and start of ANK.Results: No significant differences were identified in the ANK effectiveness and frequency of primary or secondary inefficacy for patients starting ANK within 6 months (p = 0.19, p = 0.14, and p = 0.81, respectively) or 12 months (p = 0.37, p = 0.23, and p = 0.81, respectively) since AOSD onset compared with patients starting ANK thereafter; no significant differences were identified in ANK effectiveness and primary or secondary inefficacy according with different lines of ANK treatment (p = 0.06, p = 0.19, and p = 0.13, respectively). Patients starting ANK within 6 and 12 months since AOSD onset showed a significantly quicker decrease of erythrocyte sedimentation rate and C-reactive protein than observed among patients undergoing ANK treatment after 6 and 12 months. The number of swollen joints at the 3 month follow-up visit was significantly lower among patients undergoing ANK within 6 months since AOSD onset (p = 0.01), while no significance was identified at the 6 and 12 month assessments (p = 0.23 and p = 0.45, respectively). At the 3 and 6 month visits, the number of swollen joints was significantly higher among patients previously treated with conventional and biological disease modifying anti-rheumatic drugs (DMARDs) compared with those formerly treated only with conventional DMARDs (p < 0.017).Conclusions: Clinical and therapeutic outcomes are substantially independent of how early ANK treatment is started in AOSD patients. However, a faster ANK effectiveness in controlling systemic inflammation and resolving articular manifestations may be observed in patients benefiting from IL-1 inhibition as soon as after disease onset.

Highlights

  • Adult onset Still’s disease (AOSD) is a systemic multifactorial autoinflammatory disorder considered as the adult counterpart of systemic onset juvenile idiopathic arthritis (SOJIA) [1,2,3,4,5,6]

  • One hundred and forty-one patients (48 males, 93 females) diagnosed with adult onset Still’s disease (AOSD) according with Yamaguchi criteria and treated with ANK were enrolled in the study

  • In 19 cases (13.5%) ANK represented the first treatment approach soon after NSAIDs and corticosteroids; in 93 cases (66%) ANK treatment had been introduced as first-line biologic agent soon after conventional disease modifying anti-rheumatic drugs (cDMARDs)

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Summary

Introduction

Adult onset Still’s disease (AOSD) is a systemic multifactorial autoinflammatory disorder considered as the adult counterpart of systemic onset juvenile idiopathic arthritis (SOJIA) [1,2,3,4,5,6]. According with the predominant symptoms, clinical presentation of AOSD can be classified into two main different patterns represented by a “systemic” type and a “chronic articular” type. The systemic type includes patients mainly suffering from daily spiking fevers, typical salmonlike maculopapular rash, serositis, hepatosplenomegaly, and lymphadenopathy. The chronic articular type includes patients mainly suffering from arthritis with less pronounced systemic inflammatory features. Laboratory investigations typically show an elevated white blood cell count with neutrophil predominance, increased inflammatory markers, and high levels of serum ferritin. Aim of this study was to search for any difference in the outcome of patients with adult onset Still’s disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment

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