NextGen 2024: Refractory SJIA & MAS Session Part 5

Bulleted

Text

Key Insights

An 8-year-old girl was diagnosed with Systemic Juvenile Idiopathic Arthritis (SJIA) at age 4, exhibiting typical features like fever, rash, arthritis, anemia, and thrombocytosis.
The patient's treatment course from 2021 to 2024 involved various medications, including Cyclosporine, Tocilizumab, Adalimumab, Canakinumab, Leflunomide, and Tofacitinibe, with periods of suspension and reintroduction due to gastrointestinal symptoms, secondary failure, and sourcing difficulties.
Currently, the patient maintains joint activity and experiences systemic activity after infections, managed with tofacitinib and leflunomide. Alternative treatments under consideration include different anti-JAK agents, desensitization to tocilizumab, or association with abatacept.

1/3

2/3

3/3

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?