Moreau Julie, Leurs Amélie, Ledoult Emmanuel, Sanges Sébastien, Quartier Pierre, Comont Thibault, Launay David, Hachulla Eric, Terriou Louis
Service de médecine interne et immunologie clinique, centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares de l'adulte du Nord, Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM), CHU de Lille, université de Lille, U1286-INFINITE-Institute for Translational Research in Inflammation, Inserm, 59000 Lille, France.
Service de médecine interne et immunologie clinique, centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares de l'adulte du Nord, Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM), CHU de Lille, université de Lille, U1286-INFINITE-Institute for Translational Research in Inflammation, Inserm, 59000 Lille, France.
Rev Med Interne. 2025 Jul;46(7):415-420. doi: 10.1016/j.revmed.2025.05.002. Epub 2025 May 23.
Adult-onset Still's disease (AOSD) is a systemic auto-inflammatory disorder characterized by a cytokine storm. Four major cytokines can be specifically targeted in severe, refractory or corticosteroid-dependent forms: interleukin 1 (IL-1), interleukin 6 (IL-6), interleukin 18 (IL-18), and Tumor necrosis factor alpha (TNFa). According to the French national protocol for adult-onset Still's disease, anti-IL-6 agents are the second-line of treatment after corticosteroids. We report here on the efficacy and safety of siltuximab prescribed off-label in patients with multi-refractory forms.
OBSERVATIONS/RESULTS: These 4 patients, aged 24 to 74 years old, had polycyclic and inflammatory AOSD. The introduction of siltuximab was proposed after failure or poor tolerance of several lines of treatment. A partial or complete clinico-biological response was observed in all 4 patients.
Siltuximab is a possible salvage treatment for multi-refractory AOSD.