Comparison of MS score and HScore for the diagnosis of adult-onset Still’s disease-associated macrophage activation syndrome

Abstract

Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.

Response to: ‘Comparison of MS score and HScore for the diagnosis of adult-onset Still’s disease associated macrophage activation syndrome’ by Zhanget al

“…This discordance may depend on differences in the characteristics of patient populations. As compared with the cohort of Zhang et al 1 , patients with MAS included in the study of Wang et al 6 had a lower frequency of active arthritis (31.6% vs 71.4%), central nervous system dysfunction (1.7% vs 20%) and haemorrhagic manifestations (1.7% vs 17.1%), and a higher frequency of splenomegaly (83.3% vs 22.9%). There are also remarkable diversities between the AOSD patients with MAS in the series of Zhang et al 1 and the patients with sJIA-associated MAS enrolled in our study that led to the development of the MS score.…”

“…As compared with the cohort of Zhang et al 1 , patients with MAS included in the study of Wang et al 6 had a lower frequency of active arthritis (31.6% vs 71.4%), central nervous system dysfunction (1.7% vs 20%) and haemorrhagic manifestations (1.7% vs 17.1%), and a higher frequency of splenomegaly (83.3% vs 22.9%). There are also remarkable diversities between the AOSD patients with MAS in the series of Zhang et al 1 and the patients with sJIA-associated MAS enrolled in our study that led to the development of the MS score. 2 7 Our patients had a higher frequency of hepatomegaly (70% vs 11.4%) and splenomegaly (57.9% vs 22.9%), a lower frequency of lymphadenopathy (51.4% vs 80%), and a higher median value of ferritin (5253 ng/mL vs 2000 ng/mL).…”

“…Evaluating diagnostic criteria for macrophage activation syndrome in patients with adult onset Still's disease. Response to: 'Comparison of MS score and HScore for the diagnosis of adult-onset Still's disease associated macrophage activation syndrome' by Zhang et al We thank Zhang et al 1 for their interest in our MAS/sJIA (MS) score for diagnosis of macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA). 2 Considering that sJIA and adult-onset Still's disease (AOSD) are nowadays thought to constitute a continuum of a single disease entity 3 4 and that they share a similar risk for MAS, it is worth evaluating whether the proposed diagnostic tools are suitable to detect MAS in both illnesses.…”

Diagnosis and treatment of adult-onset Still’s disease: a concise summary of the German society of rheumatology S2 guideline

“…Table 1). Most consistently, high disease activity [2,53,55,59,100,139,177,216], cytopenia (especially leukopenia) [2,15,15,100,216], raised liver enzymes [2,13,15,216], el-evated lactate dehydrogenase (LDH) [2,13,100], high ferritin serum levels [2,13,15,55,216], low fibrinogen [2,15,216], and high triglyceride values [2,122,216] have been identified. The guidelines do not include further specific recommendations concerning MAS-HLH, as guidelines on this potentially severe complication are already available [114].…”

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

“…Wang et al [19], proposed that the cut-off value should be modified from ≥ − 2.1 to ≥ − 1.08, yielding a sensitivity of 94.1% and a specificity of 95.0%. In another study, Zhang et al [20], tested the MS-score and compared with the HScore in 174 AOSD patients: HScore of ≥ 120 performed best (with a sensitivity 90.6% and specificity 89.6%), while MS score of ≥ − 0.25 performed best with a sensitivity of 75% and a specificity of 73%. They also showed with ROC analysis that HScore had a stronger ability to diagnose AOSD-associated MAS compared with MScore (AUC = 0.973 and 0.865 for HScore and MS score, respectively).…”