The clinical data of a child with refractory systemic juvenile idiopathic arthritis (sJIA) who was admitted to the Children′s Hospital, Zhejiang University School of Medicine in October 2023 were analyzed, and literature review was conducted.The patient was a 9-year-old boy, who was admitted due to recurrent fever, accompanied by rash and joint pain for more than 4 years.Four years ago, he was diagnosed with macrophage activation syndrome and sJIA.In the past 4 years, the condition recurred 3 times under treatment with glucocorticoids, Cyclosporine, and Methotrexate.He previously experienced allergic shock after 5 minutes of infusion of Tocilizumab.Due to Tocilizumab allergy, he was treated with Janus kinase inhibitors, but the disease still recurred.After informed consent was obtained from the guardian, 240 mg of Tocilizumab was administrated for desensitization once every 2 weeks and the process went smoothly.The patient has been followed up for more than 6 months and no allergic reactions had occurred.The condition was well controlled, and the Prednisone acetate tablet had been reduced to 2.5 mg orally once a day.At present, there are 10 patients undergoing desensitization treatment both domestically and internationally, including 2 males and 8 females, aged between 2.5 and 54.0 years.All of them have achieved a success in desensitization.Therefore, Tocilizumab desensitization therapy is of great significance for patients who are allergic to biologics in clinical practice.

The clinical data of a child with refractory systemic juvenile idiopathic arthritis (sJIA) who was admitted to the Children′s Hospital, Zhejiang University School of Medicine in October 2023 were analyzed, and literature review was conducted.The patient was a 9-year-old boy, who was admitted due to recurrent fever, accompanied by rash and joint pain for more than 4 years.Four years ago, he was diagnosed with macrophage activation syndrome and sJIA.In the past 4 years, the condition recurred 3 times under treatment with glucocorticoids, Cyclosporine, and Methotrexate.He previously experienced allergic shock after 5 minutes of infusion of Tocilizumab.Due to Tocilizumab allergy, he was treated with Janus kinase inhibitors, but the disease still recurred.After informed consent was obtained from the guardian, 240 mg of Tocilizumab was administrated for desensitization once every 2 weeks and the process went smoothly.The patient has been followed up for more than 6 months and no allergic reactions had occurred.The condition was well controlled, and the Prednisone acetate tablet had been reduced to 2.5 mg orally once a day.At present, there are 10 patients undergoing desensitization treatment both domestically and internationally, including 2 males and 8 females, aged between 2.5 and 54.0 years.All of them have achieved a success in desensitization.Therefore, Tocilizumab desensitization therapy is of great significance for patients who are allergic to biologics in clinical practice.

Autoinflammatory diseases (AID) in childhood is one of refractory diseases, whose pathogenesis is not completely clear. In recent years, a large number of studies have shown that NLRP3 inflammasome plays an important role in the development of AIDs in children. Inflammasome is a cytosolic multiprotein complex that can activate cysteinyl aspartate-specific protease-1 (caspase-1), to further promote the maturation and secretion of proinflammatory cytokines IL-1β and IL-18 as well as pyroptosis and regulate innate immune response. IL-1 receptor antagonist (Anakinra) and IL-1β monoclonal antibody (Canakinumab) have good therapeutic effects in children with AIDs. This article reviews the research progress of NLRP3 inflammasome in the pathogenesis of autoinflammatory diseases.

Juvenile idiopathic arthritis (JIA)is one of the most common chronic rheumatic diseases in childhood.Systemic juvenile idiopathic arthritis(SJIA) is a subtype of JIA,which may be different from other subtypes.As shown in studies,interleukin-6 (IL-6),IL-18,IFN-γ,may instead of TNF-α,play an important role in SJIA.Traditional biological agents such as TNF antagonists have limited effect in the patients with SJIA.Tocilizumab(TCZ),a humanized anti-IL-6 receptor monoclonal antibody,inhibits the binding of IL-6 with transmembrane IL-6R or soluble IL-6R,blocking the IL-6 mediated inflammation and joint destruction.This article reviews the efficacy and safety of TCZ in patients with SJIA.