Abstract
Background: Anakinra (ANA) is an effective treatment choice in patients with adult onset Still’s disease (AOSD). Variables affecting treatment survival include loss of efficacy or adverse events, but also the decision to discontinue treatment after long-term clinical remission.Objectives: Aims of this study were: (i) to assess the drug retention rate (DRR) of ANA during a long-term follow-up looking for any difference related to the line of biologic treatment, the concomitant use of conventional disease modifying anti-rheumatic drugs (cDMARDs) and the different type of AOSD (systemic versus chronic articular); (ii) to identify predictive factors of lack of efficacy, loss of efficacy, and ANA withdrawal owing to long-term remission.Methods: AOSD patients classified according with Yamaguchi criteria and treated with ANA were retrospectively enrolled in 18 Italian tertiary Centers. Demographic, laboratory, clinical and therapeutic data related to the start of ANA (baseline), the 3-month assessment and the last follow-up visit while on ANA treatment were retrospectively collected and statistically analyzed.Results: One hundred and forty-one AOSD patients (48 males, 93 females) treated with ANA for a mean period of 35.96 ± 36.05 months were enrolled. The overall DRR of ANA was 44.6 and 30.5% at the 60- and 120-month assessments, respectively, with no significant differences between: (i) biologic naïve patients and those previously treated with other biologics (log-rank p = 0.97); (ii) monotherapy and concomitant use of cDMARDs (log-rank p = 0.45); (iii) systemic and chronic articular types of AOSD (log-rank p = 0.67). No variables collected at baseline could predict primary inefficacy, while the number of swollen joints at baseline was significantly associated with secondary inefficacy (p = 0.01, OR = 1.194, C.I. 1.043–1.367). The typical AOSD skin rash was negatively related with ANA withdrawal owing to long-term remission (p = 0.03, OR = 0.224, C.I. 0.058–0.863).Conclusion: Long-term DRR of ANA has been found excellent and is not affected by different lines of biologic treatment, concomitant use of cDMARDs, or type of AOSD. The risk of losing ANA efficacy increases along with the number of swollen joints at the start of therapy, while the typical skin rash is a negative predictor of ANA withdrawal related to sustained remission.
Highlights
Adult onset Still’s disease (AOSD) is a systemic inflammatory disorder characterized by daily high-spiking fevers, evanescent salmon-colored maculopapular rash, sore throat, serositis, hepatosplenomegaly, lymphadenopathy, myalgia, arthritis, and/or arthralgia
Clinical presentation of AOSD can be distinguished into two main phenotypes: a “systemic type” characterized by predominantly systemic features including fever, rash, serositis, and organomegaly, and a “chronic articular type” with patients suffering from articular manifestations mimicking rheumatoid arthritis with a polyarticular symmetric pattern
Secondary aims of the study were: (i) to identify any impact on the drug retention rate (DRR) of ANA by the concomitant use of conventional disease modifying anti-rheumatic drugs (cDMARDs) on the DRR of ANA and by the different biologic line of ANA therapy; (ii) to assess any difference on ANA retention rate according with the different type of AOSD; (iii) to evaluate the long-term cumulative risk for loss of ANA efficacy; (iv) to assess variables related with the treatment duration of ANA; (v) to clarify which AOSD manifestations are more frequently persistent in patients suspending ANA because of lack of efficacy
Summary
Adult onset Still’s disease (AOSD) is a systemic inflammatory disorder characterized by daily high-spiking fevers, evanescent salmon-colored maculopapular rash, sore throat, serositis, hepatosplenomegaly, lymphadenopathy, myalgia, arthritis, and/or arthralgia. Laboratory investigations usually reveal leukocytosis with neutrophil predominance, increased acute-phase reactants and high levels of serum ferritin, while serum liver enzymes may be often elevated (Pouchot et al, 1991). This condition is frequently considered as the adult counterpart of systemic onset juvenile idiopathic arthritis (SOJIA) (Uppal et al, 1995; Luthi et al, 2002; Martini, 2012). Variables affecting treatment survival include loss of efficacy or adverse events, and the decision to discontinue treatment after long-term clinical remission
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