Evidence-based clinical practice guideline for adult Still’s disease

Mimura, Toshihide; Kondo, Yuya; Ohta, Akio; Iwamoto, Masahiro; Ota, Akiko; Okamoto, Nami; Kawaguchi, Yasushi; Kono, Hajime; Takasaki, Yoshinari; Takei, Shuji; Nishimoto, Norihiro; Fujimoto, Manabu; Asanuma, Yu; Matsubara, Akio; Okiyama, Naoko; Kaneko, Shunta; Takahashi, Hiroyuki; Yokosawa, Masahiro; Sumida, Takayuki

doi:10.1080/14397595.2018.1465633

Cited by 55 publications

(47 citation statements)

References 150 publications

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“…The first evidence-based clinical practice guidelines for the management of AOSD [49] emphasized the use of IL-6 inhibitors in the treatment of AOSD, but IL-1 inhibitors were judged to be "weakly recommended as a therapeutic agent against refractory AOSD" although one RCT and several case reports suggest otherwise. A proposal may be to treat a first flare of AOSD with corticosteroids and to introduce biologics in cases of steroid-refractory flares or as early as the second flare of AOSD.…”

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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“…First, we compared the clinical features between the 25 EOSD cases (the present case and the 24 cases from the literature review) and the overall AOSD group (7,(35)(36)(37)(38)(39)(40)(41)(42)(43)(44)(45) (Table 2). The mean onset age was 76.6±4.9 and 24-46 years old in the EOSD and AOSD groups, respectively.…”

Section: Literature Reviewmentioning

confidence: 99%

Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report and Review of the Literature

et al. 2020

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Adult-onset Still's disease (AOSD) usually affects young adults. Some cases of elderly-onset Still's disease (EOSD) have been reported, but its clinical features are unclear. We herein report a 74-year-old woman who developed AOSD with macrophage activation syndrome (MAS). We also reviewed 24 previous EOSD cases in patients over 70 years old and compared the findings with overall AOSD. While the clinical features were similar between the two, including the presence of MAS, disseminated intravascular coagulation was more frequent in EOSD than in AOSD. Furthermore, despite a similar frequency of glucocorticoid use, immunosuppressants and biologics were less frequently administered in EOSD than in AOSD. This report highlights the fact that typical AOSD can develop in elderly patients with some characteristic features.

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“…In resistant cases, biologic agents should be considered. Since the identification of immune pathological pathways involving mainly interleukin-1 (IL-1) beta and IL-18, but also IL-6 and TNF-alpha, agents targeting these cytokines (as anakinra -IL-1 or anti-tumor necrosis factoralpha (anti-TNF-alpha agents)) have shown to be an effective treatment in AOSD in an isolated case report and small cohorts [13].…”

Section: Discussionmentioning

confidence: 99%

Adult-Onset Still’s Disease: Typical Presentation, Delayed Diagnosis

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Costa

et al. 2020

Cureus

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Adult-onset Still's disease (AOSD) is an uncommon auto-inflammatory disease of unknown etiology, with a classical triad of fever, arthritis, and evanescent rash. Its low prevalence and lack of specific guidelines contribute to frequent delays in diagnosis and treatment. Clinical manifestations vary greatly between mainly systemic or articular symptoms and the clinical pattern between monocyclic, polycyclic, or chronic illness. Treatment options include nonsteroid anti-inflammatory drugs (NSAIDs), systemic corticoids, disease-modifying antirheumatic drugs (DMARDs), and, more recently, biological agents directed at identified immune pathological pathways like anti-interleukin-1 (IL-1) or anti-tumor necrosis factor-alpha (TNFalpha). We report a case of a 40-year-old male with persistent fever, polyarthralgia, sore throat, and rash for two weeks despite antibiotic treatment for suspected bacterial pharyngitis. During hospitalization and after extensive diagnostic workup, an AOSD diagnosis was made according to Yamaguchi's criteria and successfully managed with systemic corticoids.

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Evidence-based clinical practice guideline for adult Still’s disease

Cited by 55 publications

References 150 publications

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

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

Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report and Review of the Literature

Adult-Onset Still’s Disease: Typical Presentation, Delayed Diagnosis

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