Macrophage activation syndrome in adults: Characteristics, outcomes, and therapeutic effectiveness of etoposide-based regimen

He, Lingbo; Yao, Shuyan; Zhang, Ruoxi; Liu, Menghan; Hua, Zhengjie; Zou, Heshan; Wang, Zhao; Wang, Yini

doi:10.3389/fimmu.2022.955523

Cited by 7 publications

(3 citation statements)

References 41 publications

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“…Some studies have indicated elevated levels of TNF-α-related molecules in MAS secondary to SLE, which can serve as diagnostic markers for the transition from active SLE to MAS [ 31 ]. During the remission phase of MAS, multiple cytokines including TNF-α exhibit decreased levels compared to the active phase [ 32 ]. However, lower levels of TNF-α were found in SLE patients with MAS compared to single SLE patients in the present study.…”

Section: Discussionmentioning

confidence: 99%

Early identification of macrophage activation syndrome secondary to systemic lupus erythematosus with machine learning

Lin,

Xie,

Luo

et al. 2024

Arthritis Res Ther

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Objective The macrophage activation syndrome (MAS) secondary to systemic lupus erythematosus (SLE) is a severe and life-threatening complication. Early diagnosis of MAS is particularly challenging. In this study, machine learning models and diagnostic scoring card were developed to aid in clinical decision-making using clinical characteristics. Methods We retrospectively collected clinical data from 188 patients with either SLE or the MAS secondary to SLE. 13 significant clinical predictor variables were filtered out using the Least Absolute Shrinkage and Selection Operator (LASSO). These variables were subsequently utilized as inputs in five machine learning models. The performance of the models was evaluated using the area under the receiver operating characteristic curve (ROC-AUC), F1 score, and F2 score. To enhance clinical usability, we developed a diagnostic scoring card based on logistic regression (LR) analysis and Chi-Square binning, establishing probability thresholds and stratification for the card. Additionally, this study collected data from four other domestic hospitals for external validation. Results Among all the machine learning models, the LR model demonstrates the highest level of performance in internal validation, achieving a ROC-AUC of 0.998, an F1 score of 0.96, and an F2 score of 0.952. The score card we constructed identifies the probability threshold at a score of 49, achieving a ROC-AUC of 0.994 and an F2 score of 0.936. The score results were categorized into five groups based on diagnostic probability: extremely low (below 5%), low (5–25%), normal (25–75%), high (75–95%), and extremely high (above 95%). During external validation, the performance evaluation revealed that the Support Vector Machine (SVM) model outperformed other models with an AUC value of 0.947, and the scorecard model has an AUC of 0.915. Additionally, we have established an online assessment system for early identification of MAS secondary to SLE. Conclusion Machine learning models can significantly improve the diagnostic accuracy of MAS secondary to SLE, and the diagnostic scorecard model can facilitate personalized probabilistic predictions of disease occurrence in clinical environments.

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Section: Discussionmentioning

confidence: 99%

Early identification of macrophage activation syndrome secondary to systemic lupus erythematosus with machine learning

Lin,

Xie,

Luo

et al. 2024

Arthritis Res Ther

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“…The etoposide (VP-16)-based regimens, a kind of treatment approach for MAS, include HLH-94, HLH-04 and the modified Doxorubicin−Etoposide-Methylprednisolone (DEP) regimen ( 140 ).…”

Section: The Attractive Therapeutic Approach To Prevent Mas In Covid-19mentioning

confidence: 99%

“…found that the use of etoposide can lead to clinical improvement coinciding with a decline of systemic HMGB1, IL-18, IFN-γ, and ferritin levels ( 141 ). Although there is some evidence that this drug may aggravate the infection or make it easier to develop new infections, its benefits are obviously greater ( 140 ).…”

Section: The Attractive Therapeutic Approach To Prevent Mas In Covid-19mentioning

confidence: 99%

Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19

et al. 2023

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Nowadays, people have relaxed their vigilance against COVID-19 due to its declining infection numbers and attenuated virulence. However, COVID-19 still needs to be concern due to its emerging variants, the relaxation of restrictions as well as breakthrough infections. During the period of the COVID-19 infection, the imbalanced and hyper-responsive immune system plays a critical role in its pathogenesis. Macrophage Activation Syndrome (MAS) is a fatal complication of immune system disease, which is caused by the excessive activation and proliferation of macrophages and cytotoxic T cells (CTL). COVID-19-related hyperinflammation shares common clinical features with the above MAS symptoms, such as hypercytokinemia, hyperferritinemia, and coagulopathy. In MAS, immune exhaustion or defective anti-viral responses leads to the inadequate cytolytic capacity of CTL which contributes to prolonged interaction between CTL, APCs and macrophages. It is possible that the same process also occurred in COVID-19 patients, and further led to a cytokine storm confined to the lungs. It is associated with the poor prognosis of severe patients such as multiple organ failure and even death. The main difference of cytokine storm is that in COVID-19 pneumonia is mainly the specific damage of the lung, while in MAS is easy to develop into a systemic. The attractive therapeutic approach to prevent MAS in COVID-19 mainly includes antiviral, antibiotics, convalescent plasma (CP) therapy and hemadsorption, extensive immunosuppressive agents, and cytokine-targeted therapies. Here, we discuss the role of the therapeutic approaches mentioned above in the two diseases. And we found that the treatment effect of the same therapeutic approach is different.

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The effectiveness of the doxorubicin-etoposide-methylprednisolone regimen for adult HLH secondary to rheumatic disease

Yin,

Wang,

Wang

2024

Ann Hematol

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Macrophage activation syndrome in adults: Characteristics, outcomes, and therapeutic effectiveness of etoposide-based regimen

Cited by 7 publications

References 41 publications

Early identification of macrophage activation syndrome secondary to systemic lupus erythematosus with machine learning

Early identification of macrophage activation syndrome secondary to systemic lupus erythematosus with machine learning

Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19

The effectiveness of the doxorubicin-etoposide-methylprednisolone regimen for adult HLH secondary to rheumatic disease

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