Adult-onset Still's disease medical therapy: Difference between revisions
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{{Adult-onset Still’s disease}} | {{Adult-onset Still’s disease}} | ||
{{CMG}} ; {{AE}} | {{CMG}} ; {{AE}} {{HK}} | ||
==Overview== | ==Overview== | ||
Medical therapy in adult-onset Still's disease (AOSD) is guided by disease activity and severity. [[Corticosteroids]] are the first line therapy; [[DMARDs|Disease Modifying Anti-Rheumatic Drugs]] ([[DMARDs]]) as [[monotherapy]] or in combination with other agents may be used in refractory or complicated cases. | |||
==Medical Therapy== | ==Medical Therapy== | ||
Medical therapy in adult-onset Still's disease (AOSD) is guided by disease activity and severity. The following agents may be used in the management of AOSD:<ref name="Gerfaud-ValentinJamilloux2014">{{cite journal|last1=Gerfaud-Valentin|first1=Mathieu|last2=Jamilloux|first2=Yvan|last3=Iwaz|first3=Jean|last4=Sève|first4=Pascal|title=Adult-onset Still's disease|journal=Autoimmunity Reviews|volume=13|issue=7|year=2014|pages=708–722|issn=15689972|doi=10.1016/j.autrev.2014.01.058}}</ref> | Medical therapy in adult-onset Still's disease (AOSD) is guided by disease activity and severity. The following agents may be used in the management of AOSD:<ref name="Gerfaud-ValentinJamilloux2014">{{cite journal|last1=Gerfaud-Valentin|first1=Mathieu|last2=Jamilloux|first2=Yvan|last3=Iwaz|first3=Jean|last4=Sève|first4=Pascal|title=Adult-onset Still's disease|journal=Autoimmunity Reviews|volume=13|issue=7|year=2014|pages=708–722|issn=15689972|doi=10.1016/j.autrev.2014.01.058}}</ref> | ||
* '''1.1''' '''Adults''' | * '''1.1''' '''Adults''' | ||
** Preferred regimen (1): Prednisone PO 0.8-1 mg/kg/day | ** Preferred regimen (1): [[Prednisone]] PO 0.8-1 mg/kg/day | ||
* '''1.2 Adults (Steroid dependence)''' | * '''1.2 Adults (Steroid dependence)''' | ||
** Preferred regimen (1): Methotrexate 7.5-20 mg/week | ** Preferred regimen (1): [[Methotrexate]] 7.5-20 mg/week | ||
** Alternative regimen (1): Combination of prednisolone 20 mg/day plus oral methotrexate 5 mg/week plus cyclosporin A 5.5 mg/kg/day | ** Alternative regimen (1): Combination of [[prednisolone]] 20 mg/day plus oral [[methotrexate]] 5 mg/week plus [[Ciclosporin|cyclosporin A]] 5.5 mg/kg/day | ||
* '''1.3 Adults (Refractory AOSD)''' | * '''1.3 Adults (Refractory AOSD)''' | ||
** '''1.3.1 Chronic arthritis''' | ** '''1.3.1 Chronic arthritis''' | ||
*** Preferred regimen (1): Infliximab IV initial 3 mg/kg at 0, 2, and 6 weeks; then 3 to 6 mg/kg/dose every 8 weeks | *** Preferred regimen (1): [[Infliximab]] IV initial 3 mg/kg at 0, 2, and 6 weeks; then 3 to 6 mg/kg/dose every 8 weeks | ||
*** Preferred regimen (2): Tocilizumab IV 8 mg/kg | *** Preferred regimen (2): [[Tocilizumab]] IV 8 mg/kg | ||
*** Alternative regimen (1): Leflunomide <20 kg: 100 mg as a single dose followed by 10 mg every other day; 20 kg to 40 kg: 100 mg once daily for 2 days followed by 10 mg once daily; >40 kg: 100 mg once daily for 3 days followed by 20 mg once daily and | *** Alternative regimen (1): [[Leflunomide]] <20 kg: 100 mg as a single dose followed by 10 mg every other day; 20 kg to 40 kg: 100 mg once daily for 2 days followed by 10 mg once daily; >40 kg: 100 mg once daily for 3 days followed by 20 mg once daily and [[Azathioprine]] initial1 mg/kg/day (50 to 100 mg) given once daily or divided twice daily; after 6 to 8 weeks, may increase by 0.5 mg/kg every 4 weeks until response or up to 2.5 mg/kg/day; an adequate trial should be a minimum of 12 weeks | ||
** '''1.3.2 Systemic flares''' | ** '''1.3.2 Systemic flares''' | ||
*** Preferred regimen (1): Anakinra SC initial 1 to 2 mg/kg once daily; maximum initial dose: 100 mg; if no response after 1 to 2 weeks, may titrate up to 4 mg/kg once daily (maximum: 200 mg/day) | *** Preferred regimen (1): [[Anakinra]] SC initial 1 to 2 mg/kg once daily; maximum initial dose: 100 mg; if no response after 1 to 2 weeks, may titrate up to 4 mg/kg once daily (maximum: 200 mg/day) | ||
*** Alternative regimen (1): Canakinumab SC children ≥2 years and ≥7.5 kg and Adolescents: 4 mg/kg every 4 weeks (maximum: 300 mg per dose) | *** Alternative regimen (1): [[Canakinumab]] SC children ≥2 years and ≥7.5 kg and Adolescents: 4 mg/kg every 4 weeks (maximum: 300 mg per dose) | ||
*** Alternative regimen (2): Rilonacept | *** Alternative regimen (2): [[Rilonacept]] | ||
* '''1.4 Adults (Complicated AOSD)''' | * '''1.4 Adults (Complicated AOSD)''' | ||
** Preferred regimen (1): Methylprednisone IV | ** Preferred regimen (1): [[Methylprednisolone|Methylprednisone]] IV | ||
** Preferred regimen (2): | ** Preferred regimen (2): [[Immunoglobulin]] IV ([[Intravenous immunoglobulin|IVIG]]) 2 g/kg body weight administered in 2–5 days. | ||
** Preferred regimen (3): Cyclophosphamide PO 1 to 5 mg/kg/day (initial and maintenance dosing) | ** Preferred regimen (3): [[Cyclophosphamide]] PO 1 to 5 mg/kg/day (initial and maintenance dosing) | ||
** Preferred regimen (4): Plasma exchange therapy | ** Preferred regimen (4): [[Plasmapheresis|Plasma exchange therapy]] | ||
==References== | ==References== | ||
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{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} | ||
[[Category:Rheumatology]] | |||
Latest revision as of 13:51, 3 May 2018
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Adult-onset Still's disease |
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Differentiating Adult-onset Still’s Disease from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Template:Adult-onset Still's disease On the Web |
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American Roentgen Ray Society Images of Template:Adult-onset Still's disease |
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Template:Adult-onset Still's disease in the news |
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Risk calculators and risk factors for Template:Adult-onset Still's disease |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]
Overview
Medical therapy in adult-onset Still's disease (AOSD) is guided by disease activity and severity. Corticosteroids are the first line therapy; Disease Modifying Anti-Rheumatic Drugs (DMARDs) as monotherapy or in combination with other agents may be used in refractory or complicated cases.
Medical Therapy
Medical therapy in adult-onset Still's disease (AOSD) is guided by disease activity and severity. The following agents may be used in the management of AOSD:[1]
- 1.1 Adults
- Preferred regimen (1): Prednisone PO 0.8-1 mg/kg/day
- 1.2 Adults (Steroid dependence)
- Preferred regimen (1): Methotrexate 7.5-20 mg/week
- Alternative regimen (1): Combination of prednisolone 20 mg/day plus oral methotrexate 5 mg/week plus cyclosporin A 5.5 mg/kg/day
- 1.3 Adults (Refractory AOSD)
- 1.3.1 Chronic arthritis
- Preferred regimen (1): Infliximab IV initial 3 mg/kg at 0, 2, and 6 weeks; then 3 to 6 mg/kg/dose every 8 weeks
- Preferred regimen (2): Tocilizumab IV 8 mg/kg
- Alternative regimen (1): Leflunomide <20 kg: 100 mg as a single dose followed by 10 mg every other day; 20 kg to 40 kg: 100 mg once daily for 2 days followed by 10 mg once daily; >40 kg: 100 mg once daily for 3 days followed by 20 mg once daily and Azathioprine initial1 mg/kg/day (50 to 100 mg) given once daily or divided twice daily; after 6 to 8 weeks, may increase by 0.5 mg/kg every 4 weeks until response or up to 2.5 mg/kg/day; an adequate trial should be a minimum of 12 weeks
- 1.3.2 Systemic flares
- Preferred regimen (1): Anakinra SC initial 1 to 2 mg/kg once daily; maximum initial dose: 100 mg; if no response after 1 to 2 weeks, may titrate up to 4 mg/kg once daily (maximum: 200 mg/day)
- Alternative regimen (1): Canakinumab SC children ≥2 years and ≥7.5 kg and Adolescents: 4 mg/kg every 4 weeks (maximum: 300 mg per dose)
- Alternative regimen (2): Rilonacept
- 1.3.1 Chronic arthritis
- 1.4 Adults (Complicated AOSD)
- Preferred regimen (1): Methylprednisone IV
- Preferred regimen (2): Immunoglobulin IV (IVIG) 2 g/kg body weight administered in 2–5 days.
- Preferred regimen (3): Cyclophosphamide PO 1 to 5 mg/kg/day (initial and maintenance dosing)
- Preferred regimen (4): Plasma exchange therapy
References
- ↑ Gerfaud-Valentin, Mathieu; Jamilloux, Yvan; Iwaz, Jean; Sève, Pascal (2014). "Adult-onset Still's disease". Autoimmunity Reviews. 13 (7): 708–722. doi:10.1016/j.autrev.2014.01.058. ISSN 1568-9972.