Adult-onset Still’s disease complicated by macrophage activation syndrome during pregnancy: a case-based review

Abstract

Adult-onset Still’s disease is a rare, systemic inflammatory rheumatic disease characterized by recurrent fevers, arthritis, and an evanescent rash. One of the most serious hematologic derangements that can be seen with adult-onset Still’s disease is macrophage activation syndrome. Macrophage activation syndrome is characterized by activation of lymphocytes, resulting in a cytokine storm and hemophagocytosis in the bone marrow, along with multi-organ failure. Adult-onset Still’s disease with macrophage activation syndrome first presenting during pregnancy is exceptionally rare; here, we report two unique cases of such a presentation and review the pertinent literature. Both of our cases presented critically ill with end-organ failure, and responded to immunosuppression; fetal demise was present in one and an emergency caesarean section with a viable fetus was performed in the other patient. Maternal outcomes were favorable in both cases and both patients did well long-term with systemic therapy. Systemic immunosuppression, particularly anti-IL1 therapy, may be considered as treatment for this rare and life-threatening condition when presenting during pregnancy.