Successful Treatment of Refractory Adult-Onset Still Disease With Canakinumab

Barsotti, Simone; Neri, R; Iacopetti, Valentina; d’Ascanio, A.; Talarico, Rosaria; Tripoli, Alessandra; Bombardieri, Stefano

doi:10.1097/rhu.0000000000000082

Cited by 23 publications

(14 citation statements)

References 8 publications

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“…The excellent efficacy and clear steroid-sparing effect of tocilizumab determined in our study, concurring with the findings of a recent meta-analysis showing that AOSD patients treated with tocilizumab had a pooled remission rate of approximately 85% regardless of the phenotype [35], and of the first RCT investigating tocilizumab in AOSD [36], should make clinicians prioritize tocilizumab to anti-TNF blockers for patients with a chronic articular form of AOSD. Canakinumab showed some inconsistent results in AOSD [37][38][39] but is currently being investigated for its efficacy on AOSD's joint involvement (NCT02204293). No patient in this study received abatacept or rituximab; the data are very limited but some authors reported efficacy in patients who fail to respond to TNF, IL-1, or IL-6 inhibition [40][41][42][43][44][45].…”

Section: Discussionmentioning

confidence: 99%

Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy

et al. 2019

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Objectives: Adult-onset Still's disease (AOSD) phenotype appears to be dichotomized in systemic or chronic articular forms. As biologicals and particularly interleukin (IL)-1 and IL-6 blockers play a more and more prominent role in the treatment, their place requires clarification. This study aimed to identify factors predictive of treatment response to anakinra or tocilizumab and investigate whether the choice of biotherapy and delays in the initiation of biotherapy influenced the likelihood of steroid discontinuation. Methods: A multicenter exploratory retrospective study included all patients diagnosed with AOSD and receiving biological treatments in three regional hospitals until 2018. Clinical and biological characteristics at diagnosis and treatment-related data were collected. The nonparametric Mann-Whitney test was used to perform univariate analysis for quantitative variables, and Fisher's exact test was used for qualitative variables. Results: Twenty-seven patients were included. All but one patient achieved remission with either anakinra or tocilizumab. Treatment responses depended on disease phenotype: the presence of arthritis and a chronic articular phenotype were associated with a substantial response to tocilizumab with p = 0.0009 (OR 36 [2.6-1703]) and p = 0. 017 (OR 10 [1.22-92.6]), respectively, whereas the systemic form and the absence of arthritis were associated with a substantial response to anakinra with p = 0.0009 (OR 36 [2.6-1703]) and p = 0.017 (OR 10 [1.22-92.6]), respectively. Tocilizumab increased the likelihood of corticosteroid withdrawal (p = 0.029) regardless of delays in initiation or when it was initiated relative to other treatment in the overall therapeutic strategy. Conclusion: This study highlights the therapeutic implications of the phenotypic dichotomy of AOSD and should help us better codify AOSD treatment.

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Section: Discussionmentioning

confidence: 99%

Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy

et al. 2019

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“…The experience with anakinra in AOSD is growing, showing to be more effective than classical DMARDs [117]. Similar expectations are awaited for canakinumab, with the benefit of a longer half-life than anakinra [118,119].…”

Section: Learning From Systemic Juvenile Idiopathic Arthritismentioning

confidence: 90%

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

Colafrancesco¹,

Priori²,

Valesini³

2015

Expert Review of Clinical Immunology

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Adult-onset Still's disease (AOSD) is a known cause of fever of unknown origin. It is characterized by a triad of symptoms: spiking fever (>39°C), salmon-colored rash and arthritis/arthralgia. On a predisposing genetic background, several conditions may act as trigger for disease and among these, infectious agents are the most important. Nowadays, a dichotomous view of AOSD has been introduced which distinguishes this entity in two subsets according to the clinical features and laboratory aspects: systemic or articular. As AOSD is a diagnosis of exclusion, specific biomarkers able to facilitate differential diagnosis are needed. A number of possible biomarkers have been proposed that will be discussed in detail in this review: ferritin, IL-18, procalcitonin, s100 proteins and sCD163.

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“…Furthermore, canakinumab is also approved for treating other autoinflammatory diseases including cryopyrin-associated periodic syndromes, familial Mediterranean fever, TNF receptor-associated periodic syndrome, and hyper-IgD syndrome as well as SoJIA. Although the overall experience with canakinumab in AoSD is still limited, the reported response of systemic features and arthritis was rapid and sustained over many months to years in most patients, frequently allowing for tapering of steroids [145][146][147]. Of note, canakinumab was found highly effective for patients with AoSD who were difficult to treat, including those showing failure of NSAIDs, glucocorticoids, and other IL-1 inhibitors.…”

Section: Il-1 Inhibitionmentioning

confidence: 99%