ArticleClinical rheumatology2025
Clinical characteristics of IgG4-related retroperitoneal fibrosis in a cohort of 117 patients with idiopathic retroperitoneal fibrosis: a retrospective study.
Article in Clinical rheumatology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- Clinical features, diagnosis, and management of retroperitoneal fibrosis in a university referral hospital and literature review.Internal and emergency medicine · 2026Review
- Clinical outcomes and management of idiopathic retroperitoneal fibrosis: a case series.Annals of medicine and surgery (2012) · 2026Article
- IgG4-Related Retroperitoneal Fibrosis Presenting as Obstructive Acute Kidney Injury: A Case Report.Cureus · 2026Article
- Lymphoproliferative Disorders Mimicking Rheumatologic Disease: A Clinical Reasoning Perspective.Current allergy and asthma reports · 2025Review
- Idiopathic retroperitoneal fibrosis arising from peritoneal space: A case report and review of literature.World journal of gastroenterology · 2025Review
- Case Report: IgG4-RD-related ophthalmopathy combined with monoclonal gammopathy of undetermined significance.Frontiers in immunology · 2025Article
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Authors and funding
6 authors.
Funding
Abstract
objectiveRetroperitoneal fibrosis (RPF) is a rare condition marked by inflammation and fibrosis affecting the peritoneal and retroperitoneal soft tissues. In recent years, the identification of IgG4-related diseases has brought to light a significant association with fibrous disorders, including RPF, which were once considered independent. In this comprehensive cohort study, we performed a comparative analysis of the demographic, clinical, laboratory, histopathological, and therapeutic characteristics between patients with IgG4-related RPF and those with idiopathic retroperitoneal fibrosis (iRPF).
methodsWe performed a retrospective analysis of 117 patients diagnosed with RPF at the First Affiliated Hospital of Naval Medical University between July 2007 and July 2023.
resultsDemographic, clinical, laboratory, histopathological, and therapeutic characteristics of 70 iRPF patients and 47 IgG4-related patients were systematically compared. The IgG4-related group exhibited an older age of onset, with a predominant occurrence among adult males. Significantly elevated levels of eosinophilia and IgE were observed in the IgG4-related group. Most patients across both groups displayed elevated CRP and ESR levels. Furthermore, at the time of diagnosis, the IgG4-related group had higher serum creatinine and lower levels of complement. The most prevalent clinical manifestation in both groups was flank pain. The proportion of lymphoplasmic infiltration and storiform fibrosis in IgG4-related RPF group was significantly higher. The IgG4-related RPF group had significantly higher IgG4-positive plasma cell count, IgG4/total IgG ratio, and eosinophils count than that in iRPF group.
conclusionWe conducted a comparative analysis of demographic, clinical, laboratory, histopathological, and therapeutic differences between the iRPF patients and the IgG4-related patients. Clarifying the distinctive characteristics of these two groups will contribute to a better understanding of the condition and facilitate the development of specific treatment strategies tailored to each group. Key Points • Identification of distinct clinical features and outcomes between IgG4-related and iRPF patients in a large retrospective study.
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