Performances of the “MS-score” And “HScore” in the diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis patients

Abstract

Macrophage activation syndrome (MAS) is a devastating complication of systemic JIA (sJIA), seen in approximately 10-25% of the sJIA patients. A number of criteria have been proposed to differentiate between activation of sJIA and MAS, including HScore and the recently proposed MS-score. This is the first study comparing the performances of MS-score and HScore for the diagnosis of MAS in sJIA patients. Systemic JIA patients followed at Hacettepe University Pediatric Rheumatology Unit were included in the study. Clinical features and laboratory findings at the time when the disease was most active or patients were diagnosed with MAS were recorded retrospectively. HScore and MS-score were calculated and the diagnostic performance for MAS was compared by receiver operating characteristic (ROC) curve analysis. Seventy-one sJIA patients were included (23 MAS, 48 activation). There was no difference in age of onset (median 4.7 vs. 5.0 years) and gender (73.9% vs. 54.2%) between patients who had MAS and sJIA activation. Median MS-score and HScore were higher in the MAS group. ROC curve analysis revealed that the HScore performed slightly better in diagnosing MAS, compared with the MS-score (AUC = 0.965 and 0.901 for HScore and MS-score respectively, P < 0.001). In our cohort, the optimal cut-off for the MS score was ≥ − 1.64 (sensitivity: 91.3%; specificity: 83.8%) and for the HScore it was ≥ 162.5 (sensitivity: 91.3%; specificity: 90.2%). HScore performed slightly better than MS-score for the diagnosis of MAS in our cohort. Keywords Systemic JIA • Macrophage activation syndrome • Hscore • MS-score Abbreviations MAS Macrophage activation syndrome sJIA Systemic juvenile idiopathic arthritis AOSD Adult-onset Still's disease AUC Area under the curve ROC Receiver operating characteristic Rheumatology INTERNATIONAL Erdal Sag and Armagan Keskin contributed equally to this work.

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

“…There was disagreement about one patient who had a MS score above the cut-off level of -2.1 but did not fulfil the 2016 MAS classification criteria and another patient who fulfilled these criteria but had a MS score below the threshold. Recently, Sag et al compared the MS-score and the HScore in seventy-one sJIA patients and found that the optimal cut-off for the MS-score in their cohort was ≥ -1.64 with a sensitivity of 91.3% and a specificity of 83.8% [ 26 ], being close to the optimal cut-off value at ≥ -1.5 (sensitivity = 71.4%, specificity = 91.7%) that was found in our cohort. The difference between the MS-score ≥ −2.1 presented by Minoia et al and the cut-off value in our study [ 16 ] may depend on selection bias and the small number of patients in our study.…”

“…As the ferritin/ESR ratio only involves two tests, it is the easiest measure to identify MAS compared to the 2016 MAS classification criteria and MS score. However, a common disadvantage with all three sets of criteria is that they relate to a single time point and not changes in laboratory and clinical parameters over time [ 26 ]. Many have argued that relative changes in laboratory parameters are more useful for establishing an early MAS diagnosis than a certain threshold [ 5 , 8 , 13 , 27 ].…”

Macrophage Activation Syndrome in Children: Diagnosis and Management

“…The total score ranges between -8.4 to 41.8 with a value ≥-2.1 showing the best performance. Although the authors reported a good performance in a validation cohort, a separate study compared the MS score with the Hscore for identification of MAS in 71 patients with sJIA and found that the H-score performed slightly better [23].…”