对嗜血细胞淋巴组织细胞增多症和无嗜血细胞淋巴组织细胞增多症的成年型静止病进行全国性分析。

IF 2.4 4区 医学 Q2 RHEUMATOLOGY
JCR: Journal of Clinical Rheumatology Pub Date : 2024-08-01 Epub Date: 2024-06-04 DOI:10.1097/RHU.0000000000002100
Faria Sami, Michael Manansala, Shilpa Arora, Augustine M Manadan
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引用次数: 0

摘要

简介成人型斯蒂尔病(AOSD)是一种罕见的炎症性疾病,临床病程呈单相、间歇或慢性,其中一部分患者可能会出现嗜血细胞淋巴组织细胞增多症(HLH)等危及生命的并发症。本研究旨在描述并发 AOSD 和 HLH 的特征,并确定与院内死亡独立相关的变量:我们对2016-2019年全国住院患者抽样数据库中伴有和不伴有HLH的AOSD进行了病历回顾。我们对院内死亡进行了多变量逻辑回归分析。结果以调整后的几率比(ORadj)报告:共有5495例AOSD住院患者,其中340例(6.2%)患有HLH。AOSD和HLH合并组中有30人(9.0%)在住院期间死亡,而无HLH组中有75人(1.5%)在住院期间死亡。对AOSD住院患者进行的多变量分析显示,弥散性血管内凝血(ORadj 6.13)、肝功能衰竭(ORadj 7.16)、感染(ORadj 3.72)、呼吸衰竭(ORadj 6.89)和血栓性微血管病(ORadj 14.05)与较高的死亡几率相关。然而,HLH本身并不是AOSD人群死亡的独立预测因素:结论:HLH发生在少数AOSD住院患者中。结论:HLH发生在少数AOSD住院患者中,HLH本身并不是院内死亡的独立风险因素。弥散性血管内凝血、肝功能衰竭、感染、呼吸衰竭和血栓性微血管病与AOSD患者较高的院内死亡几率有关。更好地认识这些危及生命的并发症可改善住院预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Nationwide Analysis of Adult-Onset Still Disease With and Without Hemophagocytic Lymphohistiocytosis.

Introduction: Adult-onset Still disease (AOSD) is a rare inflammatory condition with a monophasic, intermittent, or chronic clinical course, and a subset may experience life-threatening complications such as hemophagocytic lymphohistiocytosis (HLH). This study aims to characterize concurrent AOSD and HLH and identify variables independently associated with in-hospital death.

Methods: We performed a medical records review of AOSD with and without HLH from the 2016-2019 National Inpatient Sample database. We performed a multivariable logistic regression analysis for in-hospital death. Results were reported as adjusted odds ratios (OR adj ).

Results: There were 5495 hospitalizations with AOSD, of which 340 (6.2%) had HLH. Thirty (9.0%) of the combined AOSD and HLH group died in the hospital compared with 75 (1.5%) of those without HLH. Multivariable analysis in AOSD inpatients showed that disseminated intravascular coagulation (OR adj 6.13), hepatic failure (OR adj 7.16), infection (OR adj 3.72), respiratory failure (OR adj 6.89), and thrombotic microangiopathy (OR adj 14.05) were associated with higher odds of death. However, HLH itself was not an independent predictor of mortality in AOSD population.

Conclusions: HLH occurred in a small minority of inpatients with AOSD. HLH itself was not an independent risk factor for in-hospital death. Disseminated intravascular coagulation, hepatic failure, infection, respiratory failure, and thrombotic microangiopathy were associated with higher odds of in-hospital death in AOSD. Better awareness of these life-threatening complications may improve hospital outcomes.

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来源期刊
CiteScore
3.50
自引率
2.90%
发文量
228
审稿时长
4-8 weeks
期刊介绍: JCR: Journal of Clinical Rheumatology the peer-reviewed, bimonthly journal that rheumatologists asked for. Each issue contains practical information on patient care in a clinically oriented, easy-to-read format. Our commitment is to timely, relevant coverage of the topics and issues shaping current practice. We pack each issue with original articles, case reports, reviews, brief reports, expert commentary, letters to the editor, and more. This is where you''ll find the answers to tough patient management issues as well as the latest information about technological advances affecting your practice.
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