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Internal and Emergency Medicine

, Volume 12, Issue 3, pp 287–299 | Cite as

Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease

  • Giovanni Maria Rossi
  • Rossana Rocco
  • Eugenia Accorsi Buttini
  • Chiara Marvisi
  • Augusto VaglioEmail author
IM - REVIEW

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.

Keywords

Retroperitoneal fibrosis IgG4 ANCA Hashimoto Periaortitis Hydronephrosis Vasculitis 

Abbreviations

RPF

Retroperitoneal fibrosis

IRF

Idiopathic retroperitoneal fibrosis

ANCA

Anti-neutrophil cytoplasmic antibody

IgG4-RD

IgG4-related disease

GFR

Glomerular filtration rate

US

Ultrasound

CT

Computed tomography

MRI

Magnetic resonance imaging

HBV

Hepatitis B virus

HCV

Hepatitis C virus

HIV

Human immunodeficiency virus

ANA

Anti-nuclear antibodies

18F-FDG-PET

18F-fluorodeoxyglucose positron emission tomography

AKI

Acute kidney injury

CKD

Chronic kidney disease

Notes

Compliance with ethical standards

Conflict of interest

The authors have no conflict of interest with the publication of this article.

Statement of human and animal rights

This article was written in respect of human rights.

Informed consent

Informed consent was obtained from patients whose computed tomography scans are shown in the paper.

Funding

There was no specific funding for this work.

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Copyright information

© SIMI 2017

Authors and Affiliations

  • Giovanni Maria Rossi
    • 1
  • Rossana Rocco
    • 1
  • Eugenia Accorsi Buttini
    • 1
  • Chiara Marvisi
    • 1
  • Augusto Vaglio
    • 1
    Email author
  1. 1.Nephrology UnitParma University HospitalParmaItaly

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