Refractory SJIA Patient Perspective & MAS BMT evaluation criteria

Beatriz, 22 yo, Switzerland

• ● SJIA, MAS (multiple times), LD
• ● 2018: Bone Marrow Transplant (BMT)
• ● 2024: In remission, no SJIA medications
• · Just Vitamin D and Calcium

Meds used: Steroids, Methotrexate, Actemra, Canakinumab, Anakinra, Cyclosporin, Tacrolimus

Actual treatment: Complete remission


Our Decision for BMT

• ● Shared decision with medical team - Rheumatology & BMT Teams
• ● Beatriz was feeling really sick, quality of life was getting worse everyday.
• ● ILD + Pulmonary hypertension - bad outcome in a short period of time.
• ● No other medications available, steroid dependant.
• ● Good match (unrelated donor, 9/10 match).

Note: Beatriz was HLA DRB 1*15 positive, but in 2018, we didn't know this could be related to LD.

Possible Treatment Options



Quartier P. Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches. J Clin Med . 2022;11(5):1357. Published 2022 Mar 1. doi:10.3390/jcm11051357

Evaluation for BMT depending on center & meds

• ● At least 9 BMTs patients that we know from FB had BMT since 2018
• · Different timing for BMT evaluation
• ● MAS Episodes (smoldering/full blown)
• ● Lung Disease/Persistent Arthritis/Liver reactions…
• ● HLA DRB 1*15 positive
• ● Meds availability in different countries
• ● Donor availability

Is it possible to have an algorithm or protocol for BMT evaluation in SJIA & MAS refractory cases?

Thank you!