LRG1, a novel serum biomarker for iMCD disease activity

Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by systematic inflammatory symptoms and multiorgan dysfunction caused by a cytokine storm. The current assessment of treatment response in iMCD lack sensitivity due to the heterogeneity of...

Full description

Saved in:
Bibliographic Details
Main Authors: Miao-yan Zhang, Zi-han Yang, Yu-chong Qiu, Yu-han Gao, Si-yuan Li, Yue Dang, Lu Zhang, Jian Li
Format: Article
Language:English
Published: BMC 2025-04-01
Series:Biomarker Research
Subjects:
Online Access:https://doi.org/10.1186/s40364-025-00767-1
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849726428546859008
author Miao-yan Zhang
Zi-han Yang
Yu-chong Qiu
Yu-han Gao
Si-yuan Li
Yue Dang
Lu Zhang
Jian Li
author_facet Miao-yan Zhang
Zi-han Yang
Yu-chong Qiu
Yu-han Gao
Si-yuan Li
Yue Dang
Lu Zhang
Jian Li
author_sort Miao-yan Zhang
collection DOAJ
description Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by systematic inflammatory symptoms and multiorgan dysfunction caused by a cytokine storm. The current assessment of treatment response in iMCD lack sensitivity due to the heterogeneity of clinical features. We performed proteomic analysis using Data Independent Acquisition (DIA) mass spectrometry (MS) on 33 serum samples in different disease states from 17 patients. Leucine-rich alpha-2-glycoprotein-1 (LRG1) emerged as one of the proteins with most significantly different expression, exhibiting lower levels in response to treatment. Enzyme-linked immunosorbent assay (ELISA) on a larger cohort of 146 serum samples (96 disease flare, 28 biochemical partial response, 22 biochemical complete response) from 100 iMCD patients further confirmed this association, demonstrating a significant decrease in serum LRG1 level following successful treatment. Notably, LRG1 remained elevated in patients with ongoing inflammation during siltuximab therapy when CRP failed to accurately reflect disease activity. Additionally, serum CRP/LRG1 ratio differed across iMCD subtypes, suggesting potential variations in inflammatory pathways. These findings support serum LRG1 as a valuable biomarker for iMCD disease treatment response and activity, and may provide insights into underlying disease mechanisms.
format Article
id doaj-art-e2ece620fad743d9844c1966318e6b82
institution DOAJ
issn 2050-7771
language English
publishDate 2025-04-01
publisher BMC
record_format Article
series Biomarker Research
spelling doaj-art-e2ece620fad743d9844c1966318e6b822025-08-20T03:10:10ZengBMCBiomarker Research2050-77712025-04-011311510.1186/s40364-025-00767-1LRG1, a novel serum biomarker for iMCD disease activityMiao-yan Zhang0Zi-han Yang1Yu-chong Qiu2Yu-han Gao3Si-yuan Li4Yue Dang5Lu Zhang6Jian Li7Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeAbstract Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by systematic inflammatory symptoms and multiorgan dysfunction caused by a cytokine storm. The current assessment of treatment response in iMCD lack sensitivity due to the heterogeneity of clinical features. We performed proteomic analysis using Data Independent Acquisition (DIA) mass spectrometry (MS) on 33 serum samples in different disease states from 17 patients. Leucine-rich alpha-2-glycoprotein-1 (LRG1) emerged as one of the proteins with most significantly different expression, exhibiting lower levels in response to treatment. Enzyme-linked immunosorbent assay (ELISA) on a larger cohort of 146 serum samples (96 disease flare, 28 biochemical partial response, 22 biochemical complete response) from 100 iMCD patients further confirmed this association, demonstrating a significant decrease in serum LRG1 level following successful treatment. Notably, LRG1 remained elevated in patients with ongoing inflammation during siltuximab therapy when CRP failed to accurately reflect disease activity. Additionally, serum CRP/LRG1 ratio differed across iMCD subtypes, suggesting potential variations in inflammatory pathways. These findings support serum LRG1 as a valuable biomarker for iMCD disease treatment response and activity, and may provide insights into underlying disease mechanisms.https://doi.org/10.1186/s40364-025-00767-1Leucine-rich alpha-2-glycoprotein-1 (LRG1)Idiopathic Multicentric Castleman Disease (iMCD)BiomarkerProteomics
spellingShingle Miao-yan Zhang
Zi-han Yang
Yu-chong Qiu
Yu-han Gao
Si-yuan Li
Yue Dang
Lu Zhang
Jian Li
LRG1, a novel serum biomarker for iMCD disease activity
Biomarker Research
Leucine-rich alpha-2-glycoprotein-1 (LRG1)
Idiopathic Multicentric Castleman Disease (iMCD)
Biomarker
Proteomics
title LRG1, a novel serum biomarker for iMCD disease activity
title_full LRG1, a novel serum biomarker for iMCD disease activity
title_fullStr LRG1, a novel serum biomarker for iMCD disease activity
title_full_unstemmed LRG1, a novel serum biomarker for iMCD disease activity
title_short LRG1, a novel serum biomarker for iMCD disease activity
title_sort lrg1 a novel serum biomarker for imcd disease activity
topic Leucine-rich alpha-2-glycoprotein-1 (LRG1)
Idiopathic Multicentric Castleman Disease (iMCD)
Biomarker
Proteomics
url https://doi.org/10.1186/s40364-025-00767-1
work_keys_str_mv AT miaoyanzhang lrg1anovelserumbiomarkerforimcddiseaseactivity
AT zihanyang lrg1anovelserumbiomarkerforimcddiseaseactivity
AT yuchongqiu lrg1anovelserumbiomarkerforimcddiseaseactivity
AT yuhangao lrg1anovelserumbiomarkerforimcddiseaseactivity
AT siyuanli lrg1anovelserumbiomarkerforimcddiseaseactivity
AT yuedang lrg1anovelserumbiomarkerforimcddiseaseactivity
AT luzhang lrg1anovelserumbiomarkerforimcddiseaseactivity
AT jianli lrg1anovelserumbiomarkerforimcddiseaseactivity