Adult-Onset Still's Disease

Systemic-onset rheumatoid arthritis in adults. It differs from classical rheumatoid arthritis in that it is more often marked by acute febrile onset, and generalized lymphadenopathy and hepatosplenomegaly are more prominent.

Also Known As:

Still's Disease, Adult-Onset; Adult-Onset Still Disease; Still Disease, Adult-Onset; Adult Onset Still Disease; Adult Onset Still's Disease; Adult-Onset Stills Disease; Still Disease, Adult Onset; Still's Disease, Adult Onset; Stills Disease, Adult-Onset

Networked: 732 relevant articles (63 outcomes, 39 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Arthritis (Polyarthritis)
2.	Macrophage Activation Syndrome
3.	Juvenile Arthritis (Juvenile Idiopathic Arthritis)
4.	Myocarditis (Carditis)
5.	Hemophagocytic Lymphohistiocytosis (Hemophagocytic Syndrome)

Experts

1.	Chen, Der-Yuan: 21 articles (02/2022 - 11/2004)
2.	Kim, Hyoun-Ah: 20 articles (05/2022 - 05/2012)
3.	Yang, Chengde: 19 articles (01/2022 - 10/2017)
4.	Suh, Chang-Hee: 17 articles (05/2022 - 11/2003)
5.	Shi, Hui: 17 articles (01/2022 - 10/2017)
6.	Giacomelli, Roberto: 16 articles (07/2022 - 01/2014)
7.	Ruscitti, Piero: 16 articles (07/2022 - 01/2014)
8.	Lan, Joung-Liang: 16 articles (02/2022 - 11/2004)
9.	Teng, Jialin: 16 articles (01/2022 - 01/2018)
10.	Jung, Ju-Yang: 14 articles (05/2022 - 06/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Adult-Onset Still's Disease:

1.	SteroidsIBA 03/01/2013 - "Adult-onset Still's disease was diagnosed, but treatment with steroids did not achieve remission. " 02/01/2008 - "A case of steroid-resistant adult-onset Still's disease is herein reported. " 01/01/1999 - "Steroids are needed in 80% of cases to control systemic manifestations of adult onset Still's disease. " 06/01/2016 - "Adult-onset Still's disease was diagnosed and the patient responded well to steroid therapy. " 03/01/2020 - "Learning objective: Febrile conductive disorders occurring during a systemic disorder with negative infection and auto-immunity work-up should lead to consider an adult-onset Still's disease, which can be treated and cured, especially with steroids. "
2.	tocilizumab (atlizumab)FDA Link 12/01/2018 - "The study suggests that tocilizumab is effective in adult-onset Still's disease, although the primary endpoint was not met and solid conclusion was not drawn." 06/01/2014 - "Efficacy of tocilizumab in conventional treatment-refractory adult-onset Still's disease: multicenter retrospective open-label study of thirty-four patients." 01/01/2016 - "Efficacy of Low-Dose Tocilizumab on Relapsing Adult-Onset Still's Disease." 01/01/2015 - "Two cases of adult-onset Still's disease treated with tocilizumab that achieved tocilizumab-free remission." 05/01/2014 - "Efficacy of tocilizumab in refractory adult-onset Still's disease; report of 2 cases."
3.	Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link 05/01/2013 - "Anakinra is effective in adult-onset Still's disease (AOSD) in the short term, but little is known regarding its efficacy over the long term. " 09/01/2015 - "Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review." 01/01/2020 - "Background: Aim of this study was to search for any difference in the outcome of patients with adult onset Still's disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment. " 12/01/2021 - "Anakinra for Adult-Onset Still's Disease Despite Old Age." 01/01/2020 - "Comparison of Early vs. Delayed Anakinra Treatment in Patients With Adult Onset Still's Disease and Effect on Clinical and Laboratory Outcomes."
4.	Etanercept (Enbrel)FDA Link 02/01/2006 - "Prolonged remission in adult-onset Still's disease with etanercept." 09/01/2002 - "Adult onset Still's disease: response to Enbrel." 01/01/2012 - "Angioedema of the periorbital region that developed during treatment with etanercept in a case of refractory adult-onset Still's disease." 11/01/2008 - "Etanercept as a rescue agent in patient with adult onset Still's disease complicated with congestive heart failure." 05/01/2005 - "[Application of etanercept in treatment of juvenile idiopathic arthritis and adult onset still disease]."
5.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2019 - "Managing Adult-onset Still's disease: The effectiveness of high-dosage of corticosteroids as first-line treatment in inducing the clinical remission. " 08/15/2023 - "She was ultimately diagnosed with adult-onset Still disease and responded well to treatment with systemic corticosteroids. " 01/27/2022 - "Corticosteroid-Induced Liver Injury in Adult-Onset Still's Disease." 01/01/2011 - "It is important to note that corticosteroids can conceal many diseases and they should not be considered lightly even if the patient is diagnosed with Adult onset Still's disease." 02/01/2005 - "Consensus is lacking on treatment for corticosteroid resistant adult onset Still's disease (ASD). "
6.	InterleukinsIBA 01/01/2020 - "Inhibition of interleukin (IL)-1 represents a promising treatment option in adult-onset Still's disease (AOSD). " 01/01/2018 - "Interleukin (IL)-37 has been known to play an immunosuppressive role in various inflammatory disorders, but whether it participates in the regulation of pathogenesis of adult-onset Still's disease (AOSD) has not been investigated. " 01/01/2017 - "Background: Interleukin (IL)-1 plays a crucial role in the pathogenesis of Adult onset Still's disease (AOSD). " 01/01/2012 - "Interleukin 1β (IL-1β) is emerging as a master mediator of adult onset Still's disease (AOSD) pathogenesis. " 01/01/2012 - "We report the case of a 31-year-old woman who developed adult-onset Still's disease (AOSD) with a high level of serum interleukin (IL)-18. "
7.	Infliximab (Remicade)FDA Link 01/01/2005 - "Prolonged efficacy of infliximab for refractory adult-onset Still's disease." 02/01/2004 - "Successful treatment of refractory adult-onset Still's disease with infliximab. " 10/01/2003 - "Infliximab in a case of early adult-onset Still's disease." 01/01/2002 - "A case of adult onset Still's disease treated with infliximab." 02/01/2013 - "Reversal of severe hepatitis with infliximab in adult-onset Still's disease."
8.	Interleukin-6 (Interleukin 6)IBA 01/05/2022 - "Serum concentrations of IL-6 well correlate with disease activity of adult-onset Still's disease, and blockade of IL-6 has been proven to be effective in active adult-onset Still's disease. " 01/01/2022 - "The review summarizes the importance of IL-6 in the pathogenesis of adult-onset Still's disease and clinical aspects of IL-6 inhibition from retrospective and prospective studies. " 01/01/2022 - "Interleukin-6 inhibition: a therapeutic strategy for the management of adult-onset Still's disease." 01/01/2009 - "Interleukin-6 overproduction is pathologically involved in adult onset Still's disease (AOSD). " 11/01/1996 - "Interleukin 6: a possible marker of disease activity in adult onset Still's disease."
9.	canakinumab FDA Link 01/01/2020 - "Real-Life Data on the Efficacy of Canakinumab in Patients with Adult-Onset Still's Disease." 02/25/2019 - "Correction to: Efficacy of canakinumab as first-line biologic agent in adult-onset Still's disease." 01/01/2019 - "Efficacy of canakinumab as first-line biologic agent in adult-onset Still's disease." 01/01/2020 - "Canakinumab for Treatment of Adult-Onset Still's Disease to Achieve Reduction of Arthritic Manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial." 01/01/2022 - "However, use of canakinumab in the treatment of adult-onset Still's disease and T1DM revealed negative results. "
10.	Methotrexate (Mexate)FDA LinkGeneric 07/01/2022 - "Methotrexate Failure in the Treatment of Adult-Onset Still's Disease: A Case Report." 09/01/2010 - "Use of methotrexate in adult-onset Still's disease." 02/01/1997 - "To evaluate methotrexate treatment in patients with active adult onset Still's disease (AOSD). " 02/01/1997 - "Methotrexate treatment in patients with adult onset Still's disease--retrospective study of 13 Japanese cases." 10/03/1992 - "A possible role of methotrexate in the management of adult onset Still's disease is suggested."

Therapies and Procedures

1.	Therapeutics 10/01/2012 - "However, adult-onset Still disease was diagnosed, and the patient responded well to therapy." 05/01/2012 - "To date, the treatment of adult-onset Still's disease (AOSD) has been largely empirical; therefore, this study was conducted to investigate the response to therapy and prognostic factors of AOSD. " 01/01/2023 - "[Update on Adult-Onset Still's Disease: Diagnosis, Therapy and Guideline]." 01/01/2021 - "Update on the therapy of adult-onset Still's disease with a focus on IL-1-inhibition: a systematic review." 01/01/2021 - "An Update on the Pathogenic Role of Macrophages in Adult-Onset Still's Disease and Its Implication in Clinical Manifestations and Novel Therapeutics."
2.	Plasmapheresis 10/10/1993 - "[Marked efficacy of double filtration plasmapheresis in 3 cases of adult-onset Still's disease]." 07/01/2006 - "Double-filtration plasmapheresis for resolution of corticosteroid resistant adult onset Still's disease."
3.	Peripheral Blood Stem Cell Transplantation (Peripheral Stem Cell Transplantation) 06/01/2000 - "Prolonged remission state of refractory adult onset Still's disease following CD34-selected autologous peripheral blood stem cell transplantation."
4.	Hydrotherapy (Whirlpool Bath) 04/25/2019 - "Finally, after several days and clinical dilemma, she was diagnosed with adult-onset Still's disease by the rheumatologist and was started on prednisolone, to which she showed marked improvement, and was later maintained on methotrexate and hydrotherapy. "
5.	Liver Transplantation 01/01/2022 - "Adult-onset Still's disease during pregnancy that delivered a neonate with haemophagocytic lymphohistiocytosis and severe liver failure requiring liver transplantation: A case report and literature review." 06/01/2021 - "The utility of liver transplantation to treat acute liver failure caused by adult-onset Still's disease: case reports." 06/01/2021 - "Key Points • Acute severe hepatitis in adult-onset Still's disease (AOSD) is associated with liver transplantation and/or death in, respectively, 43% and 24% of cases. " 12/01/2009 - "[Adult-onset Still's disease with liver failure requiring liver transplantation]." 11/01/2014 - "Long-term efficacy and safety of Anakinra in a patient with liver transplant due to Adult onset Still's Disease."