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IgG4-related retroperitoneal fibrosis: a newly characterized disease
Linjuan Lian1, Cong Wang1, Jian-Li Tian1
1Gerontology Department, Tianjin Medical University General Hospital, Tianjin, China.
Abstract:
Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF. IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease.
Insights
Retroperitoneal fibrosis (RPF) is a rare inflammatory condition. Immunoglobulin G4-related RPF, a subtype, requires early detection and treatment for better outcomes.
Area of Science:
- Medicine
- Pathology
- Immunology
Background:
- Retroperitoneal fibrosis (RPF) involves chronic inflammation and fibrosis in the retroperitoneum, encasing vital structures.
- RPF presents with diverse clinical manifestations due to compression of the aorta, iliac arteries, and urinary tract.
- Idiopathic retroperitoneal fibrosis (IRPF) accounts for two-thirds of cases, with unclear pathogenesis.
Purpose of the Study:
- To review the definition, epidemiology, clinical features, diagnostic criteria, treatment, and prognosis of IgG4-related RPF.
- To raise awareness of IgG4-related RPF as a newly characterized disease entity.
- To highlight the importance of early detection and treatment of IgG4-related RPF.
Main Methods:
- Literature review on retroperitoneal fibrosis and Immunoglobulin G4-related disease (IgG4-RD).
- Analysis of recent studies linking IRPF with IgG4-RD.
- Synthesis of information on clinical presentation, diagnosis, and management.
Main Results:
- A subset of IRPF patients exhibit abundant IgG4-positive plasma cell infiltration, defining IgG4-related RPF.
- IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy, leading to surgical intervention.
- IgG4-related RPF demonstrates a favorable response to glucocorticoid therapy.
Conclusions:
- IgG4-related RPF is a distinct entity within the spectrum of IgG4-RD.
- Early diagnosis and glucocorticoid treatment are crucial for managing IgG4-related RPF.
- Increased awareness of IgG4-related RPF can improve patient outcomes and prevent misdiagnosis.
