●NLRP3 inflammasomes upregulate caspase-1, cleaving pro-IL-1β and IL-18
●High ferritin correlated with systemic pattern, recurrent flares, MAS, and poor prognosis

Details PubMed ↗

Grade Anarrative review

Mechanisms, biomarkers and targets for adult-onset Still's disease

Feist E, Mitrovic S, Fautrel B · Nat Rev Rheumatol · 2018

●Comprehensive review of NLRP3 inflammasome role in AOSD pathogenesis
●IL-1β, IL-18, and IL-6 as central cytokines driving disease
●Identified biomarkers and therapeutic targets

Details PubMed ↗

Grade Bnarrative review

A comprehensive review on adult onset Still's disease

Giacomelli R, Ruscitti P, Shoenfeld Y · J Autoimmun · 2018

●MAS reported in up to 15% of AOSD patients with high mortality
●Systematic overview of clinical features, pathogenesis, and treatment

Details PubMed ↗

Grade Bcohort

Elevated expression of the NLRP3 inflammasome and its correlation with disease activity in adult-onset Still disease

Chen DY, Lan JL, Lin FJ, Hsieh TY · J Rheumatol · 2017

●NLRP3, caspase-1, and IL-1β significantly elevated in AOSD PBMCs
●NLRP3 expression correlated with disease activity

Details PubMed ↗

Grade Bcohort

Elevated serum levels of free interleukin-18 in adult-onset Still's disease

Girard C, Rech J, Brown M, et al. · Rheumatology · 2016

●Free IL-18 specifically elevated in AOSD vs other inflammatory diseases
●Supports IL-18 as potential therapeutic target

Details PubMed ↗

Grade Bcohort

IL-18 Serum Level in Adult Onset Still's Disease: A Marker of Disease Activity

Colafrancesco S, Priori R, Alessandri C, et al. · Int J Inflam · 2012

●IL-18 serum levels significantly higher in active AOSD than in inactive disease and controls
●Significant correlation between IL-18 and disease activity, ferritin, and CRP

Details PubMed ↗

Grade Bnarrative review

Ferritin in adult-onset Still's disease: just a useful innocent bystander?

Colafrancesco S, Priori R, Valesini G, et al. · Int J Inflam · 2012

●Ferritin synthesis regulated by IL-1β, IL-6, IL-18, TNF-α, IFN-γ
●Hyperferritinemia links cytokine-mediated pathogenesis to clinical biomarker

Details PubMed ↗

Grade Bcohort

Serum calprotectin as a marker for disease activity and severity in adult-onset Still's disease

Jung JY, Kim MY, Suh CH, Kim HA · J Rheumatol · 2010

●Calprotectin significantly elevated in active AOSD vs inactive disease
●Calprotectin correlates with disease activity and decreases after treatment

Details PubMed ↗

clinical

11 sources

Grade Bnarrative review

Adult-onset Still's disease: clinical aspects and therapeutic approach

Tomaras S, Goetzke CC, Kallinich T, Feist E · J Clin Med · 2021

●Immune dysregulation involves both innate and adaptive immune arms
●Advances in cytokine understanding have driven revolutionary treatment advances

Details PubMed ↗

Grade Bnarrative review

Adult-onset Still's disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies

Efthimiou P, Kontzias A, Hur P, et al. · Semin Arthritis Rheum · 2021

●Lymphadenopathy in 42.8-56.3% of AOSD patients, may mimic lymphoma
●Comprehensive review of unmet needs and targeted therapy era

Details PubMed ↗

Grade Bnarrative review

Complications of adult-onset Still's disease and their management

Efthimiou P, Kadavath S, Engel B · Expert Rev Clin Immunol · 2018

●Comprehensive review of life-threatening complications: MAS, hepatic failure, amyloidosis, cardiac
●High-dose corticosteroids and supportive measures first-line for all severe complications

Details PubMed ↗

Grade Bcohort

Macrophage activation syndrome in patients affected by adult-onset Still disease: analysis of survival rate and its causes of death

Ruscitti P, Iacono D, Ciccia F, et al. · Medicine · 2017

●MAS significantly reduced survival rate in both paediatric and adult patients
●High systemic score and ferritin independently associated with MAS occurrence

Details PubMed ↗

Grade Ccase series

Macrophage activation syndrome complicating adult onset Still's disease: a single center case series and comparison with literature

Lenert A, Yao Q · Semin Arthritis Rheum · 2016

●MAS mortality rate approximately 10-20%
●Overlap between MAS and AOSD symptoms complicates timely diagnosis

Details PubMed ↗

Grade Bnarrative review

Adult-onset Still's disease

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Seve P · Autoimmun Rev · 2014

●Comprehensive review describing AOSD as complex multigenic autoinflammatory syndrome
●Two peaks in age distribution at 15-25 and 36-46 years

Details PubMed ↗

Grade Bnarrative review

Adult-onset Still disease

Fautrel B · Best Pract Res Clin Rheumatol · 2008

●Comprehensive review of AOSD cardinal features: spiking fever, arthritis, rash, hyperleukocytosis
●Treatment strategies based on observational data rather than controlled trials

Details PubMed ↗

Grade Bnarrative review

Diagnosis and management of adult onset Still's disease

Efthimiou P, Paik PK, Bielory L · Ann Rheum Dis · 2006

●Comprehensive review of AOSD diagnosis and management
●Synthesized evidence for optimal diagnostic and therapeutic approaches

Details PubMed ↗

Grade Ccohort

Adult Still's disease. Part II. Management, outcome, and prognostic factors

Masson C, Le Loet X, Liote F, et al. · Rev Rhum Engl Ed · 1996

●23% monocyclic, 38.5% polycyclic, 38.5% chronic articular course
●Polyarthritis at onset predictive of chronic articular disease

Details PubMed ↗

Grade Ccase seriesn=21

Adult-onset Still's disease: clinical course and outcome

Cush JJ, Medsger TA Jr, Christy WC, et al. · Arthritis Rheum · 1987

●Defined four clinical course patterns: monocyclic, polycyclic, chronic articular
●Chronic articular disease had worst functional outcome

Details PubMed ↗

Grade Ccase seriesn=14

Still's disease in the adult

Bywaters EG · Ann Rheum Dis · 1971

●Original description of AOSD as adult counterpart of Still's disease in children
●Characterized 14 patients aged 17-35 with fever, rash, and arthritis

Details PubMed ↗

treatment

8 sources

Grade BRCTn=35

Canakinumab for treatment of adult-onset Still's disease to achieve reduction of arthritic manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled trial

Kedor C, Listing J, Zernicke J, et al. · Ann Rheum Dis · 2020

●67% canakinumab vs 41% placebo achieved primary endpoint (p=0.18, underpowered)
●Trial terminated prematurely due to disease rarity and EMA conditional approval

Details PubMed ↗

Grade Bcohortn=141

Long-term retention rate of anakinra in adult onset Still's disease and predictive factors for treatment response

Vitale A, Cavalli G, Ruscitti P, et al. · Front Pharmacol · 2019

●Anakinra retention rate: cumulative loss-of-efficacy risk 3.4% at 12 months, 13.5% at 60 months
●14.2% discontinued due to long-term treatment-induced remission

Details PubMed ↗

Grade Ccohort

Managing adult-onset Still's disease: the effectiveness of high-dosage of corticosteroids as first-line treatment

Ruscitti P, Cipriani P, Liakouli V, et al. · Clin Exp Rheumatol · 2019

●High-dose corticosteroids more effective than low-dose as first-line
●Associated with monocyclic pattern and corticosteroid discontinuation at 18 months

Details PubMed ↗

Grade BRCTn=27

Tocilizumab in patients with adult-onset Still's disease refractory to glucocorticoid treatment: a randomised, double-blind, placebo-controlled phase III trial

Kaneko Y, Kameda H, Ikeda K, et al. · Ann Rheum Dis · 2018

●Tocilizumab significantly improved systemic feature score (p=0.003) and glucocorticoid sparing (p=0.017)
●ACR50 at week 4: 61.5% tocilizumab vs 30.8% placebo

Details PubMed ↗

Grade Ccohortn=23

Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still's disease

Gabay C, Fautrel B, Rech J, et al. · Ann Rheum Dis · 2018

●First clinical trial of IL-18 inhibition in AOSD
●Favorable safety profile with early signs of efficacy at both 80mg and 160mg doses

Details PubMed ↗

Grade Bnarrative review

Treatment of adult-onset Still's disease: a review

Jamilloux Y, Gerfaud-Valentin M, Henry T, Seve P · Ther Clin Risk Manag · 2015

●Corticosteroids control about 60% of patients with steroid dependency in 45%
●NSAIDs effective in <20% of patients; methotrexate achieves remission in up to 70%

Details PubMed ↗

Grade BRCTn=22