Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening complication typically associated with hematologic malignancies and infections. HLH, also referred to as macrophage activation syndrome (MAS), has been recognized to complicate adult onset Still’s disease (AOSD) and systemic lupus erythematosus. Due to paucity of reported cases, there is no clear consensus concerning treatment recommendations and outcomes.

Methods: We performed a retrospective study of 5 patients with MAS complicating AOSD at the Cleveland Clinic over the past 7 years. All 5 patients underwent bone marrow biopsy. We also reviewed a total of 77 cases from various published reports and identified 49 cases with definitive evidence of MAS and with complete data. Our cohort and the historical data were compared and analyzed.

Results: We identified 5 cases (4 female, 1 male) for our cohort that satisfy both criteria for AOSD and MAS, with 3 cases simultaneously presenting with MAS and AOSD. Mean age at diagnosis of MAS was 35.8 years and mean follow-up was 14.4 months. All patients had fever, arthralgias, typical rash, and leukocytosis, with lymphadenopathy in 3/5 and sore throat in 2/5 consistent with AOSD. These patients also had MAS, with lung involvement in 2/5, renal insufficiency in 2/5 and shock in 1/5. There was significant hepatic dysfunction in all, but only 1/5 had hepatomegaly. One patient had superimposed histoplasmosis. All patients had bi-cytopenia. Besides systemic glucocorticoids, 4/5 patients received Anakinra and 2 of the patients received combination treatment with Cyclosporine. All 5 patients survived with a mean follow-up of 14.4 ± 15 months. We also reviewed 49 cases (34 female, 15 male) for the literature cohort. Mean age at diagnosis of MAS was 39.9 years and mean follow-up was 17.2 months. Compared with the historical data, the levels of ferritin and hepatic dysfunction were similarly higher, and triglycerides were less elevated in our cohort, while fibrinogen was normal in both. Soluble IL-2 receptor level was significantly higher as well.

Conclusion: MAS can be a serious complication of AOSD and may coexist with its onset. In conjunction with literature, this study of a relatively largest case series suggests that treatment with a triple combination of an IL-1 receptor antagonist, a calcineurin inhibitor and systemic glucocorticoids may have favorable outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/macrophage-activation-syndrome-complicating-adult-onset-stills-disease-single-center-experience-and-literature-review/