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A clinical analysis of hemophagocytic syndrome secondary to autoimmune diseases

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收录情况： ◇ SCIE

作者：
Liu Yang[1,2] * ; Li Qian[3] ; Su Yazhen[1,2] ; Che Guozhu[1,2] ; Liu Ying[1,2] ; Qiao Pengyan[1,2] ; Liu Sumiao[1,2] ; Xu Ke[1,2] ;
单位： [1]Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Third Hospital of Shanxi Medical University, Taiyuan, China. [2]Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. [3]Department of Rheumatology and Immunology, Heping Hospital Affiliated to Changzhi Medical College, Changzhi, China.
出处：
DOI： 10.46497/ArchRheumatol.2023.9728
ISSN：

关键词： Autoimmune diseases hemophagocytic syndrome macrophage activation syndrome

摘要：
This study aimed to analyze the differences of etiologies and clinical features between patients with autoimmune-associated hemophagocytic syndrome (AAHS) and those with other underlying diseases of hemophagocytic syndrome (HPS).The retrospective study was performed with 130 HPS patients (70 males, 60 females; mean age: 50.4±18.1 years; range, 13 to 85 years) between January 1st, 2011, and April 1st, 2022. The patients fulfilled at least five of the eight criteria proposed by the Histiocytosis Society in 2004. The underlying diseases related to HPS were divided into four categories: autoimmune, infection, malignancy and idiopathic diseases. And the clinical manifestations, laboratory examinations, treatments, and prognosis were analyzed respectively.Nineteen (14.6%) patients had AAHS, 45 (34.6%) had infection-associated HPS, 57 (43.8%) had malignancy-associated HPS, and nine (6.9%) had idiopathic HPS. The most common symptoms of HPS were unremitting fever in 123 (94.6%) of 130 patients and splenomegaly in 92 (70.8%). All patients manifested a decline of at least two lineages of hematopoietic cells. The absolute values of T cells and B cells of AAHS were significantly higher than that of malignancy-associated HPS. The levels of soluble CD25 (interleukin-2 receptor) of AAHS were the lowest among all-cause HPS (p<0.05). The all-cause mortality rate of hospitalized patients with HPS was 46.2%. The patients with AAHS had a better prognosis compared to other etiologies (odds ratio [OR]=0.091, 95% confidence interval [CI]: 0.011-0.775, p=0.028). Epstein-Barr virus infection (OR=4.761, 95% CI: 1.619-14.004, p=0.005) and pulmonary involvement (OR=4.555 95% CI: 1.524-13.609, p=0.007) were independent predictors of poor outcome in HPS. Thrombocytopenia (OR=0.978, 95% CI: 0.968-0.999, p=0.040) had a boundary effect on prognosis.Patients with HPS secondary to autoimmune disease have better outcomes compared to patients complicated with Epstein-Barr virus infection or pulmonary involvement.Copyright © 2023, Turkish League Against Rheumatism.

基金：
This work was supported by grants from the National Natural Science Foundation (81871292) and the Key Research and Development (R&D) Projects of Shanxi Province (201803D31136).
语种：
外文
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中科院(CAS)分区：
出版当年[2022]版：
大类 | 4 区 医学
小类 | 4 区 风湿病学
最新[2025]版：
大类 | 4 区 医学
小类 | 4 区 风湿病学
JCR分区：
出版当年[2021]版：
Q4 RHEUMATOLOGY
最新[2023]版：
Q4 RHEUMATOLOGY

影响因子： 最新[2023版] 最新五年平均 出版当年[2021版] 出版当年五年平均 出版前一年[2020版] 出版后一年[2022版]

第一作者：
第一作者单位： [1]Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Third Hospital of Shanxi Medical University, Taiyuan, China. [2]Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
通讯作者：
通讯机构： [1]Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Third Hospital of Shanxi Medical University, Taiyuan, China. [2]Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
推荐引用方式(GB/T 7714)：
Liu Yang,Li Qian,Su Yazhen,et al.A clinical analysis of hemophagocytic syndrome secondary to autoimmune diseases[J].ARCHIVES OF RHEUMATOLOGY.2023,38(3):406-418.doi:10.46497/ArchRheumatol.2023.9728.
APA：
Liu Yang,Li Qian,Su Yazhen,Che Guozhu,Liu Ying...&Xu Ke.(2023).A clinical analysis of hemophagocytic syndrome secondary to autoimmune diseases.ARCHIVES OF RHEUMATOLOGY,38,(3)
MLA：
Liu Yang,et al."A clinical analysis of hemophagocytic syndrome secondary to autoimmune diseases".ARCHIVES OF RHEUMATOLOGY 38..3(2023):406-418

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