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Adult-Onset Still's Disease

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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: 343 relevant articles (33 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Chen, Der-Yuan: 10 articles (01/2015 - 11/2004)
2.	Lan, Joung-Liang: 10 articles (01/2015 - 11/2004)
3.	Chen, Yi-Ming: 6 articles (01/2015 - 10/2009)
4.	Hsieh, Chia-Wei: 6 articles (01/2015 - 12/2007)
5.	Chen, Hsin-Hua: 5 articles (01/2015 - 10/2009)
6.	Lin, Chi-Chen: 5 articles (01/2015 - 10/2009)
7.	Efthimiou, Petros: 5 articles (03/2014 - 06/2007)
8.	Yoo, Dae-Hyun: 5 articles (10/2012 - 02/2004)
9.	Nishimoto, Norihiro: 4 articles (05/2011 - 05/2005)
10.	Hsieh, Tsu-Yi: 4 articles (12/2007 - 11/2004)

Drugs and Biologics

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

1.	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. " 05/01/2008 - "Effective treatment of steroid refractory adult-onset Still's disease with anakinra." 09/01/2015 - "Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review." 06/01/2007 - "Anakinra treatment in patients with adult-onset Still's disease is fast, effective, safe and steroid sparing: experience from an uncontrolled trial." 04/01/2015 - "We report a 46-year-old white woman with adult-onset Still's disease (AOSD) treated with anakinra, a IL-1B receptor antagonist. "
2.	tocilizumab FDA Link 06/01/2014 - "Efficacy of tocilizumab in conventional treatment-refractory adult-onset Still's disease: multicenter retrospective open-label study of thirty-four patients." 01/01/2015 - "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." 05/01/2005 - "Clinical efficacy of tocilizumab in RA, soJIA, adult-onset Still's disease or CD patients has been discussed in this review. " 05/01/2015 - "The effect of tocilizumab on preventing relapses in adult-onset Still's disease: A retrospective, single-center study."
3.	TNFR-Fc fusion protein (etanercept)FDA Link 02/01/2006 - "Prolonged remission in adult-onset Still's disease with etanercept." 01/01/2014 - "We herein report the case of an 84-year-old man with steroid-dependent adult-onset Still's disease (AOSD) whose daily steroid dose was successfully tapered after etanercept treatment. " 01/01/2014 - "Steroid-sparing effects of etanercept in a patient with steroid-dependent adult-onset Still's disease." 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."
4.	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." 06/01/2006 - "Administration of infliximab was discontinued for treatment of adult onset Still's disease, and steroid levels were reduced following double-membrane filtration plasma exchange. "
5.	SteroidsIBA 03/01/2013 - "Adult-onset Still's disease was diagnosed, but treatment with steroids did not achieve remission. " 01/01/1999 - "Steroids are needed in 80% of cases to control systemic manifestations of adult onset Still's disease. " 01/01/2013 - "We present a case of adult onset Still's disease associated with myocarditis requiring the use of invasive ventilation, in which the patient responded well to systemic steroids." 08/01/2010 - "To evaluate the efficacy of antiinflammatory agents, steroids, immunosuppressants, and biologic agents in patients with adult-onset Still's disease (AOSD) who have either chronic articular disease or nonchronic disease. " 05/01/2009 - "Anakinra seems promising in the treatment of childhood or adult onset Still disease, after the failure of both high dose steroids and methotrexate. "
6.	Interleukin-18 (Interleukin 18)IBA 01/01/2014 - "Interleukin-18 as an efficient marker for remission and follow-up in patients with inactive adult-onset Still's disease." 03/01/2001 - "This study was undertaken to determine levels of IL-18 and IL-18 binding inhibition in the blood of patients with adult-onset Still's disease (ASD). " 10/01/2012 - "To investigate natural killer (NK) cell proportions, NK cell cytotoxicity, and interleukin 18 (IL-18) expression, in patients with adult-onset Still's disease (AOSD). " 06/01/2012 - "Transient impairment of NK cell function in an infant born to a mother with adult-onset Still's disease: perinatal effect of maternal IL-18." 01/01/2012 - "IL-18 Serum Level in Adult Onset Still's Disease: A Marker of Disease Activity."
7.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 05/01/2009 - "TNFalpha blockers might be an option in refractory ANCA-associated vasculitis, sarcoïdosis, adult onset Still disease, Behçet disease, AA amyloïdosis and TRAPS. " 11/01/2006 - "Decrease of proteinuria in a patient with adult-onset Still's disease and glomerulonephritis after anti-TNFalpha therapy." 01/01/2005 - "To evaluate the efficacy of the anti-TNFα (Tumor Necrosis Factor alpha) drugs in a variety of pathologic conditions such as psoriatic arthritis, undifferentiated entheso-arthritides, chronic inflammatory bowel diseases, adult onset Still's disease, Behcet disease. " 10/01/2010 - "Exacerbation of adult-onset Still's disease, possibly related to elevation of serum tumor necrosis factor-alpha after etanercept administration." 09/01/2005 - "Adalimumab (anti-TNF-alpha) therapy to improve the clinical course of adult-onset Still's disease: the first case report."
8.	Immunoglobulins (Immunoglobulin)IBA 10/01/2009 - "[Adult-onset Still's disease with pulmonary and cardiac involvement and response to intravenous immunoglobulin]." 02/01/2009 - "High levels of immunoglobulins have been observed in patients with the active stage of adult onset Still's disease (AOSD), and high-dose intravenous immunoglobulin treatment has decreased the disease activity of AOSD. " 01/01/1999 - "Precise mechanisms of action of intravenous immunoglobulin in adult onset Still's disease remain unclear. " 11/01/2012 - "Reversible posterior leukoencephalopathy syndrome in a case of adult onset Still's disease with concurrent thrombotic thrombocytopenic purpura: response to high dose immunoglobulin infusions." 11/01/2007 - "Comment on: Adult-onset Still's disease and myocarditis: successful treatment with intravenous immunoglobulin and maintenance of remission with etanercept."
9.	Interleukin-6 (Interleukin 6)IBA 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." 05/01/2011 - "A considerable number of case reports and preclinical studies have shown the benefit of IL-6 blockade therapy in various IMIDs such as systemic lupus erythematosus, adult-onset Still disease, Takayasu arthritis, polyarteritis nodosa, systemic sclerosis, reactive arthritis, dermatomyositis, and polymyositis. " 05/01/2015 - "To investigate the clinical effectiveness of tocilizumab (TCZ), a monoclonal antibody against the interleukin-6 receptor, in preventing relapse in patients with adult-onset Still's disease (AOSD). " 03/01/2014 - "A novel therapeutic approach in pulmonary arterial hypertension as a complication of adult-onset Still's disease: targeting IL-6."
10.	Antirheumatic Agents (DMARD)IBA 10/01/2012 - "To study the efficacy of anakinra versus disease-modifying antirheumatic drugs (DMARD) in refractory adult-onset Still's disease (AOSD). " 09/01/2011 - "Adult-onset Still disease (AOSD) is an inflammatory condition of unknown etiology that responds to glucocorticosteroids and disease-modifying antirheumatic drugs, particularly methotrexate. " 12/01/2011 - "Adult-onset Still's disease (AOSD) is empirically treated with nonsteroidal anti-inflammatory drugs, corticosteroids, conventional disease-modifying antirheumatic drugs, tumor necrosis factor-blocking agents or anakinra. "

Therapies and Procedures

1.	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."
2.	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."
3.	Plasma Exchange 01/01/2008 - "Successful treatment with plasma exchange in adult-onset Still's disease with hyper-IL-18-naemia and hyperallergic state." 07/10/2011 - "[Case report: a case of adult-onset Still's disease with thrombotic microangiopathy-like disorder successfully treated by plasma exchange and anti-TNF therapy]." 01/01/2010 - "A case report of successful treatment with plasma exchange for adult-onset Still's disease with autoimmune hepatitis." 06/01/2006 - "Administration of infliximab was discontinued for treatment of adult onset Still's disease, and steroid levels were reduced following double-membrane filtration plasma exchange. " 01/01/2013 - "Successful treatment of glucocorticoid and cyclosporine refractory adult-onset Still's disease complicated with hemophagocytic syndrome with plasma exchange therapy and tocilizumab : a case report."
4.	Transplants (Transplant) 01/01/2014 - "Immune reconstitution inflammatory syndrome in a patient with adult-onset Still's disease: graft-versus-host-like skin reaction with possible asymptomatic human herpes virus reactivation during steroid tapering." 11/01/2014 - "Long-term efficacy and safety of Anakinra in a patient with liver transplant due to Adult onset Still's Disease."
5.	Biological Therapy 11/01/2014 - "Adult onset Still's disease (AOSD) in the era of biologic therapies: dichotomous view for cytokine and clinical expressions." 09/01/2012 - "Pleuritis is a red flag for adult-onset Still's disease which may require biologic therapies."