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Clinical Rheumatology

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01-08-2020 | Glucocorticoid | Original Article

Macrophage activation syndrome associated with adult-onset Still’s disease: a multicenter retrospective analysis

Authors: Ran Wang, Ting Li, Shuang Ye, Wenfeng Tan, Cheng Zhao, Yisha Li, Chun de Bao, Qiong Fu

Published in: Clinical Rheumatology | Issue 8/2020

Abstract

Objectives

To explore the clinical features, treatments, and prognostic factors of adult-onset Still’s disease (AOSD)-associated macrophage activation syndrome (MAS), we conducted a multicenter retrospective clinical study of AOSD-associated MAS patients.

Methods

AOSD patients were collected from six tertiary hospitals in China. Medical charts were reviewed and clinical information was recorded and analyzed.

Results

There were 447 AOSD patients enrolled into this retrospective clinical study. Among them, 55 were diagnosed with MAS. Liver dysfunction was the most reliable predictive factor for the screening of MAS in AOSD patients (OR = 75.744, 95%CI = 23.015–249.284, p < 0.0001). In multivariate analysis, clinical features including platelets < 100 × 10⁹/L (OR = 9.546, p = 0.005), aspartate transaminase (AST) > 120 U/L (OR = 25.853, p < 0.0001), triglycerides > 3 mmol/L (OR = 12.9833, p = 0.011)), ferritin > 1500 ng/mL (OR = 5.513, p = 0.050), as well as hemophagocytosis in bone puncture (OR = 18.132, p = 0.001) were highly associated with the occurrence of MAS. The mortality rate of total AOSD patients was 4.47%, MAS was the main cause of death in AOSD patients (OR = 11.705, 95%CI = 4.783–28.647, p < 0.0001). PLT ≤ 100 × 10⁹/L (p = 0.0001), fibrinogen < 1.5 g/L (p = 0.0286), splenomegaly (p = 0.0002), and liver dysfunction (p = 0.0008) highly suggested poor prognosis.

Conclusion

MAS occurrence is the major cause of death in AOSD patients. Notable liver dysfunction, as well as splenomegaly, low number of platelets or neutrophils, high levels of serum ferritin, and reduced level of fibrinogen are risk factors for poor outcome.

Key Points

• This is a multicenter retrospective study of AOSD-associated MAS with large number of cases.

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Title: Macrophage activation syndrome associated with adult-onset Still’s disease: a multicenter retrospective analysis
Authors: Ran Wang
Ting Li
Shuang Ye
Wenfeng Tan
Cheng Zhao
Yisha Li
Chun de Bao
Qiong Fu
Publication date: 01-08-2020
Publisher: Springer International Publishing
Keyword: Glucocorticoid
Published in: Clinical Rheumatology / Issue 8/2020
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-020-04949-0

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Macrophage activation syndrome associated with adult-onset Still’s disease: a multicenter retrospective analysis

Abstract

Objectives

Methods

Results

Conclusion

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