Pia Elkjær Høeg, Mia Glerup, Birgitte Mahler, Christian Høst, Troels Herlin
{"title":"评估系统性幼年特发性关节炎患者的巨噬细胞激活综合征:单中心经验。","authors":"Pia Elkjær Høeg, Mia Glerup, Birgitte Mahler, Christian Høst, Troels Herlin","doi":"10.1155/2022/1784529","DOIUrl":null,"url":null,"abstract":"<p><strong>Objectives: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":51715,"journal":{"name":"International Journal of Rheumatology","volume":null,"pages":null},"PeriodicalIF":2.3000,"publicationDate":"2022-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9348923/pdf/","citationCount":"3","resultStr":"{\"title\":\"Evaluation of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis: A Single Center Experience.\",\"authors\":\"Pia Elkjær Høeg, Mia Glerup, Birgitte Mahler, Christian Høst, Troels Herlin\",\"doi\":\"10.1155/2022/1784529\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objectives: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>\",\"PeriodicalId\":51715,\"journal\":{\"name\":\"International Journal of Rheumatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":2.3000,\"publicationDate\":\"2022-07-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9348923/pdf/\",\"citationCount\":\"3\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International Journal of Rheumatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1155/2022/1784529\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2022/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q2\",\"JCRName\":\"RHEUMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Rheumatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1155/2022/1784529","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2022/1/1 0:00:00","PubModel":"eCollection","JCR":"Q2","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
Evaluation of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis: A Single Center Experience.
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.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
