Evaluation of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis: A Single Center Experience

Abstract

Objectives. Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile arthritis (sJIA), and early diagnosis is critical for survival. The objective of this study was to evaluate the 2016 MAS classification criteria in a Danish sJIA cohort and to compare different sets of criteria for the early identification of MAS including the HLH-2004 diagnostic guidelines, MS score, and the ferritin/ESR ratio. Methods. Data was extracted from medical charts of 32 patients with sJIA from a single Danish paediatric rheumatology center diagnosed between January 2014 and June 2021. Patients who met the 2016 MAS classification criteria were classified as having MAS. From a receiver operating characteristic (ROC) plot, the area under the curve (AUC) was calculated for the prediction of patients with MAS according to the 2016 MAS classification criteria using either MS score or the ferritin/ESR ratio. Results. Of the cohort, eight (25%) patients were classified as having MAS according to the 2016 MAS classification criteria compared to only three (9.4%) patients fulfilling the HLH-2004 diagnostic guidelines, all of which had recurrent MAS. The ferritin/ESR ratio showed the highest sensitivity (100%) but the lowest specificity (72.2%). In comparison, the MS score had a higher specificity (90.9%) for the identification of MAS according to the 2016 classification criteria. In our cohort, the most optimal cut-off point for the ferritin/ESR ratio was ≥19.4 (sensitivity: 100%, specificity: 72.2%) and ≥ -1.5 for the MS score (sensitivity: 71.4%, specificity: 91.7%), respectively. Conclusion. The 2016 MAS classification criteria were a valuable tool in the discrimination of sJIA with and without MAS. The HLH-2004 diagnostic guidelines showed the lowest sensitivity, ferritin/ESR ratio, and the lowest specificity compared to the MS score where an acceptable high sensitivity and specificity was found.

Systemic juvenile idiopathic arthritis: frequency and long-term outcome in Western Australia

“…Median age at S-JIA diagnosis (6.5 years) falls with the reported range of 4.5 to 12 years in cohort studies [ 16 , 17 ]. Our cohort contained a higher proportion of girls as in the original paper by Sir George Still [ 18 ] and the US cohort study [ 19 ] although other papers report gender equivalence [ 16 , 17 ].…”

“…Median age at S-JIA diagnosis (6.5 years) falls with the reported range of 4.5 to 12 years in cohort studies [ 16 , 17 ]. Our cohort contained a higher proportion of girls as in the original paper by Sir George Still [ 18 ] and the US cohort study [ 19 ] although other papers report gender equivalence [ 16 , 17 ]. In hospital management included a high rate of intravenous corticosteroid therapy (48%) and use of a biological agent in almost a fifth of patients, in line with current and proposed therapy strategies to rapidly reduce inflammatory burden [ 20 , 21 ].…”

Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis: A single-center, 10-year-long experience

“…Macrophage activation a variant HLH, most often accompanies systemic juvenile idiopathic arthritis (sJIA) [8] and systemic lupus erythematosus (SLE) [9], but can be encountered in a wide range of autoimmune disorders [10]. The only child in our series with autoimmune disease who was diagnosed with HLH suffered from ANCA-positive systemic vasculitis.…”

Biomarkers in Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome and Their Importance in COVID Era

“…In discriminating between sJIA with and without MAS, the importance of the ferritin/ESR ratio, as well as the early identification of significant changes in serum ferritin and neutrophil, leukocyte, and platelet counts, is essential in the early stages of MAS, as evidenced by other recent studies [ 231 ].…”