علاج التهاب المفاصل اليفعي مجهول السبب ذي البدء الجهازي مع متلازمة تنشيط الضامة

References

DOI: 10.1002/art.42037

• Up to 40% of cases of systemic JIA are associated with MAS, a secondary hemophagocytic syndrome that is a life-threatening complication requiring urgent recognition and treatment.
• MAS presents with fevers, high ferritin levels, cytopenias, elevated liver enzyme levels, low fibrinogen levels, and high triglyceride levels.
• IL-1 or IL-6 inhibitors are conditionally recommended over calcineurin inhibitors alone to achieve inactive disease and resolution of MAS for systemic JIA with MAS.
• There is no preferred agent.
• Glucocorticoids are conditionally recommended as part of initial treatment of systemic JIA with MAS.
• Systemic glucocorticoids may be necessary for severely ill patients because they can have a rapid onset of action.
• Biologic DMARDs combined with glucocorticoids and calcineurin inhibitors may be necessary to control MAS in some patients.