Systemic Juvenile Idiopathic Arthritis: Resistant disease, UK experience

Paul Brogan Professor of vasculitis

and consultant paediatric rheumatologist

. … Changing therapeutic landscape


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

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

Resistant disease: treatment




Thank you

Front. Pediatr., Vol 11 (2023)

https://doi.org/10.3389/fped.2023.1218312