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    NextGen 2024: Refractory SJIA & MAS Session Part 2

    NextGen 2024: Refractory SJIA & MAS Session Part 2

    S
    @SJIA_Foundation
    8 Followers
    5 months ago 312

    Presentation from Dr. Paul brogan: Systemic Juvenile Idiopathic Arthritis: resistant disease, UK experience

    Systemic Juvenile Idiopathic Arthritis: 
Resistant disease, UK experience
Paul Brogan
Professor of vasculitis 
and consultant paediatric rheumatologist
    1/7
    .…Changing therapeutic landscape
2015 “interim” 
NHS Clinical 
Commissioning 
Policy 
2005 2021
Methotrexate - Woo P et al. Arthritis Rheum. 2000;43(8):1849–57.
Anakinra ANAJIS trial - Quartier et al. Ann Rheum Dis 2011;70:747–754.
TocilizumabTENDER Trial - De Benedetti et al. N Engl J Med 2012;367:2385-95.
Canakinumab - Ruperto et al; N Engl J Med. 2012 Dec 20;367(25):2396-406.
Emapalumab - Ann Rheum Dis. 2023 Jun;82(6):857-865.
    2/7
    Summary - GOSH experience of sJIA (n=76)
Overall sJIA outcome good – at last review (median 4.7 years, 0.2-16);
• 88% - Clinically Inactive Disease (CID)
• 84% - Glucocorticoid free CID
• 32% - Remission
Biologic treatment < 3 months results in significantly better outcome at 1 year
• Clinically inactive disease 90% versus 53%, p = 0.002
No difference in longer term outcomes (CID and GC free CID) in relation to 
timing of bDMARD in our series
Foley C et al; Front. Pediatr., Vol 11 (2023) 
https://doi.org/10.3389/fped.2023.1218312
    3/7
    Complications: 2005-2021
Macrophage Activation Syndrome
• 46% (n=35/76) – At least one episode of 
suspected MAS throughout disease course 
• Treatments received: 
• Glucocorticoids
• Anakinra
• Ciclosporin 
• Etoposide 
• Emapalumab (clinical trial)
Resistant disease: Bone marrow 
transplant
• Four patients 
• All achieved clinically inactive disease
sJIA – Lung disease 
• One patient (1%)
Mortality 
• No patients died during study period (2005-2021)
• One patient died from MAS, 7-months after data 
collection completed
Underdiagnosed
    4/7
    Resistant disease: treatment 
Arthritis
Systemic 
inflammation
Smouldering/recurrent 
MAS
sJIA-Lung disease
Lots of options 
as per poly JIA
Anakinra
Tocilizumab
Canakinumab
JAK-inhib
Ciclosporin
MTX
Glucocorticoids
Anakinra
Tocilizumab
Canakinumab
Emapalumab
JAK-inhib
Ciclosporin
Glucocorticoids
Etoposide
Allo-HSCT
Anti-IL18?
Anti-IL18/IL1?
Anakinra
Tocilizumab
Canakinumab
Emapalumab?
JAK-inhib
Ciclosporin
Glucocorticoids
Allo-HSCT
Anti-IL18?
Anti-IL18/IL1?
    5/7
    Thank you
Front. Pediatr., Vol 11 (2023) 
https://doi.org/10.3389/fped.2023.1218312
    6/7
    sJIA/Still’s 
Switch to SC or IV 
Tocilizumab10
 Active systemic 
features?
YES
NO
Anakinra8,11,13,19,26,49,50,51 2-8 mg/kg/day SC 
(usual max 100 mg/day), *usually with 
glucocorticoids: 
• Prednisolone 1-2 mg/kg/day
• +/- IV methylprednisolone 10-30 mg/kg/day 
(max 1000 mg) for 3 doses 
• Add MTX for active 
arthritis if not on already
• Switch to anti-TNF 
• Switch to Canakinumab12
(compassionate use) or consider 
other **biologic48
• Consider addition of Ciclosporin
• If fails, consider allogeneic HSCT
Inadequate response 
after 3 months
Inadequate response 
after 3 months
Inadequate response after 
3-6 months
    7/7

    NextGen 2024: Refractory SJIA & MAS Session Part 2

    • 1. Systemic Juvenile Idiopathic Arthritis: Resistant disease, UK experience Paul Brogan Professor of vasculitis and consultant paediatric rheumatologist
    • 2. .…Changing therapeutic landscape 2015 “interim” NHS Clinical Commissioning Policy 2005 2021 Methotrexate - Woo P et al. Arthritis Rheum. 2000;43(8):1849–57. Anakinra ANAJIS trial - Quartier et al. Ann Rheum Dis 2011;70:747–754. TocilizumabTENDER Trial - De Benedetti et al. N Engl J Med 2012;367:2385-95. Canakinumab - Ruperto et al; N Engl J Med. 2012 Dec 20;367(25):2396-406. Emapalumab - Ann Rheum Dis. 2023 Jun;82(6):857-865.
    • 3. Summary - GOSH experience of sJIA (n=76) Overall sJIA outcome good – at last review (median 4.7 years, 0.2-16); • 88% - Clinically Inactive Disease (CID) • 84% - Glucocorticoid free CID • 32% - Remission Biologic treatment < 3 months results in significantly better outcome at 1 year • Clinically inactive disease 90% versus 53%, p = 0.002 No difference in longer term outcomes (CID and GC free CID) in relation to timing of bDMARD in our series Foley C et al; Front. Pediatr., Vol 11 (2023) https://doi.org/10.3389/fped.2023.1218312
    • 4. Complications: 2005-2021 Macrophage Activation Syndrome • 46% (n=35/76) – At least one episode of suspected MAS throughout disease course • Treatments received: • Glucocorticoids • Anakinra • Ciclosporin • Etoposide • Emapalumab (clinical trial) Resistant disease: Bone marrow transplant • Four patients • All achieved clinically inactive disease sJIA – Lung disease • One patient (1%) Mortality • No patients died during study period (2005-2021) • One patient died from MAS, 7-months after data collection completed Underdiagnosed
    • 5. Resistant disease: treatment Arthritis Systemic inflammation Smouldering/recurrent MAS sJIA-Lung disease Lots of options as per poly JIA Anakinra Tocilizumab Canakinumab JAK-inhib Ciclosporin MTX Glucocorticoids Anakinra Tocilizumab Canakinumab Emapalumab JAK-inhib Ciclosporin Glucocorticoids Etoposide Allo-HSCT Anti-IL18? Anti-IL18/IL1? Anakinra Tocilizumab Canakinumab Emapalumab? JAK-inhib Ciclosporin Glucocorticoids Allo-HSCT Anti-IL18? Anti-IL18/IL1?
    • 6. Thank you Front. Pediatr., Vol 11 (2023) https://doi.org/10.3389/fped.2023.1218312
    • 7. sJIA/Still’s Switch to SC or IV Tocilizumab10 Active systemic features? YES NO Anakinra8,11,13,19,26,49,50,51 2-8 mg/kg/day SC (usual max 100 mg/day), *usually with glucocorticoids: • Prednisolone 1-2 mg/kg/day • +/- IV methylprednisolone 10-30 mg/kg/day (max 1000 mg) for 3 doses • Add MTX for active arthritis if not on already • Switch to anti-TNF • Switch to Canakinumab12 (compassionate use) or consider other **biologic48 • Consider addition of Ciclosporin • If fails, consider allogeneic HSCT Inadequate response after 3 months Inadequate response after 3 months Inadequate response after 3-6 months


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