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Retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding retroperitoneal structures, such as aorta, vena cava, ureters and psoas muscle.
Biomarker and diagnostic research for IgG4-related retroperitoneal fibrosis has been reported in the published literature.
Estimated prevalence: 1-9 in 100,000 (Uncommon).
6 clinical trials registered, 5 recruiting. Interventions under study include other interventions and drug therapy. Pipeline includes 1 PHASE4, 1 PHASE2. Research is primarily sponsored by academic and government institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT04312854](https://clinicaltrials.gov/study/NCT04312854) |
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 8:23 AM UTC
Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about IgG4-related retroperitoneal fibrosis
A Prospective Cohort Study of IRPF in China
— |
Peking Union Medical College Hospital |
RECRUITING |
[NCT04314323](https://clinicaltrials.gov/study/NCT04314323) | National Registry of IRPF in China | — | Peking Union Medical College Hospital | RECRUITING |
[NCT06065852](https://clinicaltrials.gov/study/NCT06065852) | National Registry of Rare Kidney Diseases | — | UK Kidney Association | RECRUITING |
[NCT04047576](https://clinicaltrials.gov/study/NCT04047576) | Study of Sirolimus in Idiopathic Retroperitoneal Fibrosis | PHASE2 | Peking University International Hospital | RECRUITING |
[NCT05428826](https://clinicaltrials.gov/study/NCT05428826) | Early Discontinuation of Steroid Treatment in Negative FDG-PET/CT Patients With Idiopathic Retroperitoneal Fibrosis | PHASE4 | Assistance Publique - Hôpitaux de Paris | RECRUITING |
113 publications have been identified in PubMed for IgG4-related retroperitoneal fibrosis. Research spans Case Report / Case Series (33%), Review / Meta-Analysis (29%), and Epidemiology / Natural History (18%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 37 | 33% |
Research summaries | 32 | 29% |
Disease patterns and progression | 20 | 18% |
Testing and diagnosis research | 11 | 10% |
Clinical study results | 7 | 6% |
Laboratory research | 2 | 2% |
New treatment approaches | 2 | 2% |
Other research | 1 | 1% |
Roberts SJ (2026). [PMID: 41846629](https://pubmed.ncbi.nlm.nih.gov/41846629/). *Cureus*. [Case Report / Case Series]
Thiele T (2026). [PMID: 41504870](https://pubmed.ncbi.nlm.nih.gov/41504870/). *Z Rheumatol*. [Diagnostic / Biomarker]
Niksińska A (2026). [PMID: 42217044](https://pubmed.ncbi.nlm.nih.gov/42217044/). *Rheumatol Int*. [Epidemiology / Natural History]
Logronio AJ (2026). [PMID: 42146996](https://pubmed.ncbi.nlm.nih.gov/42146996/). *BJR Case Rep*. [Case Report / Case Series]
Moore KE (2026). [PMID: 41582886](https://pubmed.ncbi.nlm.nih.gov/41582886/). *Mod Rheumatol Case Rep*. [Case Report / Case Series]
Zheng T (2026). [PMID: 41794762](https://pubmed.ncbi.nlm.nih.gov/41794762/). *Eur J Med Res*. [Clinical Trial Publication]
Ren L (2026). [PMID: 41609521](https://pubmed.ncbi.nlm.nih.gov/41609521/). *Ultrasound quarterly*. [Diagnostic / Biomarker]
Bayerl C (2026). [PMID: 40810802](https://pubmed.ncbi.nlm.nih.gov/40810802/). *Eur J Nucl Med Mol Imaging*. [Diagnostic / Biomarker]
Calheiros A (2026). [PMID: 41777981](https://pubmed.ncbi.nlm.nih.gov/41777981/). *Cureus*. [Case Report / Case Series]
Jarai MZ (2026). [PMID: 42170116](https://pubmed.ncbi.nlm.nih.gov/42170116/). *Cureus*. [Case Report / Case Series]
AI-curated news mentioning IgG4-related retroperitoneal fibrosis
Updated Sep 7, 2026
A recent case report highlights simultaneous idiopathic mediastinal and retroperitoneal fibrosis in a patient who presented with acute respiratory failure. This literature review adds to the understanding of this rare condition and its clinical implications.
Recent research identifies novel biomarkers for idiopathic retroperitoneal fibrosis through autoantibody reactome analysis. These findings could enhance diagnostic and therapeutic strategies for this rare condition.