Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of&amp;nbsp;its&amp;nbsp;efficacy and safety

Hong, Dongsheng; Yang, Zhihai; Shu-yin, Han; Liang, Xiaoyang; Ma, Kuifen; Zhang, Xingguo

doi:10.2147/dddt.s73428

Cited by 29 publications

(13 citation statements)

References 50 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These articles included 1 RCT [18, 33, 61], 1 post hoc analysis of pooled study results [31], 1 prospective open-label trial [54], 5 nationwide surveys [60, 66, 71, 73, 74], 17 retrospective observational studies or case series [6, 8, 57–59, 62–64, 67, 68, 70, 72, 76, 78], 1 meta-analysis [69], and 1 comprehensive review [77]. A total of 60 case reports, mostly related to the use of anakinra were also identified; 7 case reports described the use of canakinumab in AOSD [79–85], and 3 case reports documented the use of rilonacept in AOSD [83, 86, 87]; the complete list of case reports can be viewed in Additional file 1: Table S3 [27, 59, 79–134].…”

Section: Resultsmentioning

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

View full text Add to dashboard Cite

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.

show abstract

Section: Resultsmentioning

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

View full text Add to dashboard Cite

show abstract

“…Although the efficacy of ANA in AOSD has been described by several case-reports and case-series, due to the disease’s rarity, large randomized control trials (RCT) are still lacking. A meta-analysis published in 2014 identified eight studies, including one RCT (Nordström et al, 2012) demonstrating that ANA seems to be effective in improving AOSD manifestations and in reducing mean steroid dosage over time in patients refractory to conventional therapies (Hong et al, 2014). That meta-analysis, which considered all studies published between 2010 and 2014 (sample size ranging from 6 to 28 patients for a total of 134 patients receiving ANA treatment (100 mg/day), showed an overall and complete remission rate of 81.66 and 66.75%, respectively, thus supporting the efficacy of ANA in more than half of the patients studied.…”

Section: Discussionmentioning

confidence: 99%

Response to Interleukin-1 Inhibitors in 140 Italian Patients with Adult-Onset Still’s Disease: A Multicentre Retrospective Observational Study

et al. 2017

View full text Add to dashboard Cite

Background: Interleukin (IL)-1 plays a crucial role in the pathogenesis of Adult onset Still’s disease (AOSD).Objectives: To evaluate the efficacy and safety of anakinra (ANA) and canakinumab (CAN) in a large group of AOSD patients.Methods: Data on clinical, serological features, and concomitant treatments were retrospectively collected at baseline and after 3, 6, and 12 months from AOSD patients (Yamaguchi criteria) referred by 18 Italian centers. Pouchot’s score was used to evaluate disease severity.Results: One hundred forty patients were treated with ANA; 4 were subsequently switched to CAN after ANA failure. The systemic pattern of AOSD was identified in 104 (74.2%) of the ANA-treated and in 3 (75%) of the CAN-treated groups; the chronic-articular type of AOSD was identified in 48 (25.8%) of the ANA-treated and in 1 (25%) of the CAN-treated groups. Methotrexate (MTX) was the most frequent disease modifying anti-rheumatic drug (DMARD) used before beginning ANA or CAN [91/140 (75.8%), 2/4 (50%), respectively]. As a second-line biologic DMARD therapy in 29/140 (20.7%) of the patients, ANA was found effective in improving all clinical and serological manifestations (p < 0.0001), and Pouchot’s score was found to be significantly reduced at all time points (p < 0.0001). No differences in treatment response were identified in the ANA-group when the patients were stratified according to age, sex, disease pattern or mono/combination therapy profile. ANA primary and secondary inefficacy at the 12-month time point was 15/140 (10.7%) and 11/140 (7.8%), respectively. Adverse events (AEs) [mainly represented by in situ (28/47, 59.5%) or diffuse (12/47, 25.5%) skin reactions and infections (7/47, 14.8%)] were the main causes for discontinuation. Pouchot’s score and clinical and serological features were significantly ameliorated at all time points (p < 0.0001) in the CAN-group, and no AEs were registered during CAN therapy. Treatment was suspended for loss of efficacy only in one case (1/4, 25%).Conclusion: This is the largest retrospective observational study evaluating the efficacy and safety of IL-1 inhibitors in AOSD patients. A good response was noted at 3 months after therapy onset in both the ANA- and CAN-groups. Skin reaction may nevertheless represent a non-negligible AE during ANA treatment.

show abstract

“…The recombinant antagonist of the IL-1 receptor, anakinra, has been used successfully in the refractory AOSD with systemic symptoms [18, 23]. Furthermore, anakinra is not associated with an increased risk of adverse events [24] and has been successfully used in patients with HLH complicating AOSD [18]. …”

Section: Discussionmentioning

confidence: 99%

Adult Onset Still’s Disease Associated withMycoplasma pneumoniaeInfection and Hemophagocytic Lymphohistiocytosis

Agnihotri

Ruff

Gotterer

et al. 2016

Case Reports in Medicine

View full text Add to dashboard Cite

Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated. She had hypoalbuminemia, elevated liver enzymes, a very high serum ferritin level, positive anti-Mycoplasma pneumonia IgG and IgM antibodies, and normal rheumatoid factor and anti-nuclear antibodies. The chest X-ray showed diffuse bilateral infiltrates. Bone marrow biopsy revealed hemophagocytosis. The patient was treated with azithromycin, methylprednisolone, and anakinra and was discharged home on cyclosporine and prednisone. This case highlights that patients can develop features of both AOSD and HLH at the beginning of the disease and early diagnosis and treatment increase the likelihood of recovery.

show abstract

Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety

Cited by 29 publications

References 50 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

Response to Interleukin-1 Inhibitors in 140 Italian Patients with Adult-Onset Still’s Disease: A Multicentre Retrospective Observational Study

Adult Onset Still’s Disease Associated withMycoplasma pneumoniaeInfection and Hemophagocytic Lymphohistiocytosis

Contact Info

Product

Resources

About