Macrophage activation syndrome-associated adult onset Still disease treatment: a scoping review of case reports and case series.

OBJECTIVE

One of the most lethal complications of adult-onset Still disease (AOSD) is macrophage activation syndrome, which can be present later in the case of uncontrolled AOSD or concurrently with the first diagnosis of AOSD. Up to the present, there has not been a standard guideline of treatment for AOSD associated with macrophage activation syndrome (MAS). Therefore, this systematic scoping review aimed to examine the available literature and provide an overview of treatment based on the available literature, mostly case reports and case series.

METHODS

A search strategy combined terms for AOSD, MAS, hemophagocytic lymphohistiocytosis, and treatment from PubMed, Web of Science, Embase, and Scopus databases from inception to June 2024. Studies were included and excluded by two independent authors. The quality of the studies was assessed. A qualitative and quantitative narrative synthesis of the results was conducted based on the study design.

RESULTS

Among 611 articles identified, 204 duplicates were removed, and 171 studies were included for full-text screening. A total of 83 articles met the inclusion criteria, with 69 case reports and 14 case series. Overall, mortality was 12.07%. There were more females (72.41%) than males (27.59%). Most required more than one medical treatment or treatment modality (80.17%), and 18.97% of all cases utilized monotherapy, mainly systemic corticosteroids. Others required combined therapy, ranging from two to five therapies.

CONCLUSION

Systemic glucocorticoids should be administered as first-line treatment concomitantly with either a conventional or biologic agent to suppress cytokine storms. The findings from this systematic review can be effectively applied to patients who are cared for and provide alternatives in circumstances where patients are resistant to general treatment.