التهاب المفاصل اليفعي الجهازي مع متلازمة تنشيط البلاعم عند عدم تحقيق السيطرة بالعلاج الخطي الأول

References

• 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.
• As response to individual agents is variable, switching among and between IL-1 and IL-6 inhibitors when needed due to lack of efficacy or poor tolerability is appropriate.
• 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.