Evidence map›Paper›PMID 38783316›Full record

ArticlePediatric rheumatology online journal2024

Single center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases.

Shuoyin Huang, Yingying Liu, Wu Yan, Tonghao Zhang, Panpan Wang, Meifang Zhu, Xiaohua Zhang, Peng Zhou, Zhidan Fan, Haiguo Yu

Abstract read
In one paragraph

Article in Pediatric rheumatology online journal, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Review
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Shuoyin Huang *Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Yingying Liu *Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Wu YanDepartment of Child Health Care, Children's Hospital of Nanjing Medical University, Nanjing, China.
Tonghao ZhangDepartment of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Panpan WangDepartment of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Meifang ZhuDepartment of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Xiaohua ZhangDepartment of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Peng ZhouDepartment of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.
Zhidan Fan *Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China. zhidanfan@njmu.edu.cn.
Haiguo Yu *Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China. haiguo_yu@njmu.edu.cn.ORCID http://orcid.org/0000-0001-8255-9390

Funding

National Natural Science Foundation of China 81202345National Natural Science Foundation of China 81771762, and 82271838
6 · The paper itself

Abstract

backgroundMacrophage activation syndrome (MAS), an example of secondary hemophagocytic lymphohistiocytosis, is a potentially fatal complication of rheumatic diseases. We aimed to study the clinical and laboratory characteristics, treatment schemes, and outcomes of different rheumatic disorders associated with MAS in children. Early warning indicators of MAS have also been investigated to enable clinicians to make a prompt and accurate diagnosis.

methodsFifty-five patients with rheumatic diseases complicated by MAS were enrolled between January 2017 and December 2022. Clinical and laboratory data were collected before disease onset, at diagnosis, and after treatment with MAS, and data were compared between patients with systemic juvenile idiopathic arthritis (sJIA), Kawasaki disease (KD), and systemic lupus erythematosus (SLE). A random forest model was established to show the importance score of each variable with a significant difference.

resultsMost (81.8%) instances of MAS occurred during the initial diagnosis of the underlying disease. Compared to the active stage of sJIA, the platelet count, erythrocyte sedimentation rate, and fibrinogen level in sJIA-MAS were significantly decreased, whereas ferritin, ferritin/erythrocyte sedimentation rate, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and D-dimer levels were significantly increased. Ferritin level, ferritin/erythrocyte sedimentation rate, and platelet count had the greatest predictive value for sJIA-MAS. The level of IL-18 in the sJIA-MAS group was significantly higher than in the active sJIA group, whereas IL-6 levels were significantly lower. Most patients with MAS were treated with methylprednisolone pulse combined with cyclosporine, and no deaths occurred.

conclusionsThrombocytopenia, ferritin levels, the ferritin/erythrocyte sedimentation rate, and elevated aspartate aminotransferase levels can predict the occurrence of MAS in patients with sJIA. Additionally, our analysis indicates that IL-18 plays an important role in the pathogenesis of MAS in sJIA-MAS.

Indexed as

Arthritis, JuvenileMacrophage Activation SyndromeAdolescentBlood SedimentationChildChild, PreschoolFemaleFerritinsHumansLupus Erythematosus, SystemicMaleMucocutaneous Lymph Node SyndromePlatelet CountRetrospective StudiesFerritinsFerritinInterleukin (IL)-18Kawasaki diseaseMacrophage activation syndromeRheumatic diseasesSystemic juvenile idiopathic arthritisSystemic lupus erythematosus

Identifiers

PMID38783316
PMCPMC11112803

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.