Development and Role in Therapy of Canakinumab in Adult-Onset Still’s Disease

Galozzi, Paola; Baggio, Chiara; Bindoli, Sara; Oliviero, Francesca; Sfriso, Paolo

doi:10.3389/fphar.2018.01074

Cited by 14 publications

(14 citation statements)

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“…These reports demonstrate that canakinumab is effective even in these difficult-to-treat patients who are likely to have more severe disease. Based on our systematic review of the literature and on the most recent review by Galozzi and colleagues [20], there are only 24 published cases of patients with AOSD who were treated with canakinumab. These reports show efficacy in reducing both systemic and articular manifestations, while reducing the need for corticosteroids.…”

Section: Resultsmentioning

confidence: 99%

“…Specifically, there is only one randomized controlled trial (RCT) on the use of anakinra in patients with AOSD [18], and data supporting the efficacy/safety of canakinumab are only available from two RCTs performed in patients with SJIA [19]. Based on the similarities with SJIA, the European Medicines Agency (EMA) has now approved the use of canakinumab in patients with AOSD [20]. To address the lack of evidence-based guidance on the management of AOSD, a panel of ten Italian physicians with expertise in the management of Still’s disease convened a meeting in January 2018 with the goal of addressing unresolved issues and reaching consensus about the management of patients with AOSD using IL-1 inhibitors.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

et al. 2019

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BackgroundAdult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Because of its rarity, clinical trials are inherently small and often uncontrolled. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion.MethodsA panel of 10 experts (9 rheumatologists and 1 pediatrician) was established. The first step was dedicated to a comprehensive literature review and development of statements. Two separate literature searches were performed on the MEDLINE (Pubmed), EMBASE, and BIOSIS databases through April 2018 to identify (1) differences and similarities between AOSD and pediatric Still’s disease (systemic juvenile idiopathic arthritis [SJIA]) and (2) the efficacy and safety of IL-1 inhibitors in AOSD treatment. In the second step, the statements were submitted in a Delphi process to a panel of 67 rheumatologists. Consensus threshold was set at 66%: positive, > 66% of voters selected scores 3 to 5; negative, > 66% of voters selected scores 1 or 2. In the third step, the voting results were analyzed, and the statements were finalized.ResultsEleven statements were developed. Forty-six of 67 rheumatologists (72%) participated in the Delphi process. A positive consensus was reached after the first round of voting and was full (> 95%) on the majority of statements. A large consensus was achieved in considering AOSD and SJIA as the same disease. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. Because of the lack of head-to-head comparisons, a different profile of efficacy among IL-1 inhibitors could not be established. There was a large consensus that failure of the first IL-1 inhibitor does not preclude response to another one. The lack of studies comparing early versus late treatment did not allow to draw conclusions; however, data from SJIA suggest a better response in early treatment.ConclusionsThe Delphi method was used to develop recommendations that we hope will help clinicians in the management of patients with AOSD refractory to conventional therapies.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

et al. 2019

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“…With an incidence of 0.16-0.62 per 100,000 population worldwide, adult-onset Still's disease (AOSD) is a rare multisystem inflammatory disease that usually affects young adults (1). Although the exact pathogenesis behind AOSD remains unknown, a combination of genetic and environmental factors seems to be involved (2).…”

Section: Introductionmentioning

confidence: 99%

18F-Fluorodeoxyglucose Positron Emission Tomography and Computed Tomography With Magnetic Resonance for Diagnosing Adult-Onset Still's Disease

et al. 2020

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The objective of the study was to assess the advantages of 18 F-fluorodeoxyglucose (FDG) positron emission tomography and computed tomography with magnetic resonance (PET/CT-MR) in diagnosing and monitoring patients with adult-onset Still's disease (AOSD). Methods: Participants in this retrospective case-control study underwent whole-body 18 F-FDG-PET/CT-MR imaging. All PET scans were qualitatively and semiquantitatively analyzed using standardized uptake values (SUVs) normalized to liver uptake, i.e., we calculated the ratio (SUVr) between the minimum, maximum, and mean SUVs for different organs and tissues and the mean SUV for the liver. Disease activity scores were assessed using Pouchot's criteria. Results: Eighteen patients diagnosed with AOSD and 24 controls (non-AOSD patients diagnosed with solid tumors, excluding lymphomas) were considered. A total of 38 PET/MR and nine PET/CT scans were analyzed. AOSD patients had higher SUVr than controls. All SUVr differed significantly between the patient and control group for bone marrow, and for the spleen, the only difference lacking statistical significance concerned the ratio of the minimum SUV for spleen to the mean SUV for liver. Though limited in number, AOSD responders to therapy showed lower uptakes during the period monitored. No correlations were found between Pouchot's scores and SUVr. Conclusion: Our data revealed higher spleen and bone marrow 18 F-FDG uptakes on PET/CT and PET/MR images in AOSD patients than in controls. Together with clinical examinations and laboratory data, PET/CT and PET/MR seemed more reliable than Pouchot's score in assessing disease activity.

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“…The available data indicate that canakinumab is effective and well tolerated for treating AOSD. 45 , 47 – 49 , 110 , 142 , 143 A study has shown that treatment of adult patients with AOSD and active joint involvement with canakinumab led to a clinically significant improvement of outcome measures including DAS28-ESR, DAS28-CRP, ACR30, ACR50 and ACR70 and remission, compared with placebo. It was difficult to complete the recruitment of this trial due to the rarity and severity of the disease as well as the conditional approval of canakinumab for AOSD by the EMA; therefore, the study was terminated prematurely.…”

Section: Adult Onset Still’s Diseasementioning

confidence: 99%

New insights on multigenic autoinflammatory diseases

Efthimiou¹,

Petryna

Nakasato

et al. 2022

Therapeutic Advances in Musculoskeletal

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Autoinflammatory diseases are disorders of the innate immune system, which can be either monogenic due to a specific genetic mutation or complex multigenic due to the involvement of multiple genes. The aim of this review is to explore and summarize the recent advances in pathogenesis, diagnosis, and management of genetically complex autoinflammatory diseases, such as Schnitzler’s syndrome; adult-onset Still’s disease; synovitis, acne, pustulosis, hyperostosis, osteitis syndrome/chronic recurrent multifocal osteomyelitis/chronic non-bacterial osteomyelitis; Adamantiades-Behçet’s disease; Yao syndrome; and periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome. The PubMed database was screened for relevant articles using free text words and specific search strings. The search was limited to English-language articles, reporting the results of studies in humans, published through March 2021. Evidence from literature suggest that these rare multigenic autoinflammatory diseases can present with different clinical features and the diagnosis of these diseases can be challenging due to a combination of nonspecific manifestations that can be seen in a variety of other conditions. Diagnostic delays and disease complications may occur due to low disease awareness and the lack of pathognomonic markers. The pathogeneses of these diseases are complex and in some cases precise pathogenesis is not clearly understood. Conventional treatments are commonly used for the management of these conditions, but biologics have shown promising results. Biologics targeting proinflammatory cytokines including IL-1, IL-6, TNF-α, IL-17A and IL-18 have been shown to ameliorate signs and symptoms of different multigenic autoinflammatory diseases.

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Development and Role in Therapy of Canakinumab in Adult-Onset Still’s Disease

Cited by 14 publications

References 53 publications

Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

Management of adult-onset Still’s disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts

18F-Fluorodeoxyglucose Positron Emission Tomography and Computed Tomography With Magnetic Resonance for Diagnosing Adult-Onset Still's Disease

New insights on multigenic autoinflammatory diseases

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