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Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis

Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF...

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Autores principales: Wang, Kunkun, Wang, Zhenfan, Zeng, Qiaozhu, Zhu, Lijuan, Gao, Jingyuan, Wang, Ziqiao, Zhang, Shanshan, Yang, Fei, Shen, Danhua, Wang, Yi, Liu, Yanying
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7891782/
https://www.ncbi.nlm.nih.gov/pubmed/33600452
http://dx.doi.org/10.1371/journal.pone.0245601
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author Wang, Kunkun
Wang, Zhenfan
Zeng, Qiaozhu
Zhu, Lijuan
Gao, Jingyuan
Wang, Ziqiao
Zhang, Shanshan
Yang, Fei
Shen, Danhua
Wang, Yi
Liu, Yanying
author_facet Wang, Kunkun
Wang, Zhenfan
Zeng, Qiaozhu
Zhu, Lijuan
Gao, Jingyuan
Wang, Ziqiao
Zhang, Shanshan
Yang, Fei
Shen, Danhua
Wang, Yi
Liu, Yanying
author_sort Wang, Kunkun
collection PubMed
description Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People’s Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.
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spelling pubmed-78917822021-03-01 Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis Wang, Kunkun Wang, Zhenfan Zeng, Qiaozhu Zhu, Lijuan Gao, Jingyuan Wang, Ziqiao Zhang, Shanshan Yang, Fei Shen, Danhua Wang, Yi Liu, Yanying PLoS One Research Article Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People’s Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes. Public Library of Science 2021-02-18 /pmc/articles/PMC7891782/ /pubmed/33600452 http://dx.doi.org/10.1371/journal.pone.0245601 Text en © 2021 Wang et al http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Article
Wang, Kunkun
Wang, Zhenfan
Zeng, Qiaozhu
Zhu, Lijuan
Gao, Jingyuan
Wang, Ziqiao
Zhang, Shanshan
Yang, Fei
Shen, Danhua
Wang, Yi
Liu, Yanying
Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
title Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
title_full Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
title_fullStr Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
title_full_unstemmed Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
title_short Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
title_sort clinical characteristics of igg4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7891782/
https://www.ncbi.nlm.nih.gov/pubmed/33600452
http://dx.doi.org/10.1371/journal.pone.0245601
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