NextGen 2024: Refractory SJIA & MAS Session Part 5

    NextGen 2024: Refractory SJIA & MAS Session Part 5

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    @SJIA_Foundation
    8 Followers
    6 months ago 361

    AIAI Summary

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    Key Insights

    LVL, girl, 8 years old
SJIA diagnosis at 4 years old with typical features 
(fever, rash, arthritis, anemia, thrombocytosis)
    1/3
    Tofacitinibe
Corticosteroids (including several 
pulse therapies) throughout the 
period, since 2020
Leflunomide suspended 
due to gastrointestinal 
symptoms
Jan
Currently - joint activity is maintained, 
using tofacitinib and leflunomide, with 17 
active joints and episodes of systemic 
activity after infections.
Hb 11.2*; leuc – 15.410 (N 65%; L 26,9%); 
Plaq 528,000*, ESR – 89*, CRP - 10,87*; 
Fibrinogen - 637*; LDH 409; Ferritine - 343
Jan
Cyclosporine 
Adalimumab 
suspended
due to 
secondary 
failure 
Fev
Jun
Tocilizumab
2021
Jul
Cyclosporine 
suspended
Ago
Tocilizumab suspended 
(anaphylactic shock)
Methotrexate initiated
Out
Adalimumab
2022
Methotrexate
suspended
Abril
Canakinumab
Jun
Leflunomide
Ago
2023
Jul
Canakinumabe 
suspended due to 
difficulty in sourcing the 
medication and 
persistence of systemic 
symptoms. Return to 
Leflunomide
2024
Nov
Jul
MAS - pulse therapy and 
cyclosporine
March
MAS – IVIG, pulse 
therapy and 
cyclosporine
    2/3
    Potential alternatives:
A different anti-JAK agent?
Another attempt at desensitization to tocilizumab?
Association with abatacept?
    3/3

    NextGen 2024: Refractory SJIA & MAS Session Part 5

    • 1. LVL, girl, 8 years old SJIA diagnosis at 4 years old with typical features (fever, rash, arthritis, anemia, thrombocytosis)
    • 2. Tofacitinibe Corticosteroids (including several pulse therapies) throughout the period, since 2020 Leflunomide suspended due to gastrointestinal symptoms Jan Currently - joint activity is maintained, using tofacitinib and leflunomide, with 17 active joints and episodes of systemic activity after infections. Hb 11.2*; leuc – 15.410 (N 65%; L 26,9%); Plaq 528,000*, ESR – 89*, CRP - 10,87*; Fibrinogen - 637*; LDH 409; Ferritine - 343 Jan Cyclosporine Adalimumab suspended due to secondary failure Fev Jun Tocilizumab 2021 Jul Cyclosporine suspended Ago Tocilizumab suspended (anaphylactic shock) Methotrexate initiated Out Adalimumab 2022 Methotrexate suspended Abril Canakinumab Jun Leflunomide Ago 2023 Jul Canakinumabe suspended due to difficulty in sourcing the medication and persistence of systemic symptoms. Return to Leflunomide 2024 Nov Jul MAS - pulse therapy and cyclosporine March MAS – IVIG, pulse therapy and cyclosporine
    • 3. Potential alternatives: A different anti-JAK agent? Another attempt at desensitization to tocilizumab? Association with abatacept?


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