Standardized approach to idiopathic retroperitoneal fibrosis: a comprehensive review of the literature

ABSTRACT

Background

Idiopathic retroperitoneal fibrosis (iRPF) is a rare fibro-inflammatory disease, characterized by inflammation of the abdominal aorta and its surrounding structures. The exact pathophysiology remains unclear. Diagnosis is often troublesome due to the non-specific and highly variable clinical presentation. Standardized treatment protocols are lacking.

Objective

This article presents a review on iRPF, addressing clinical and diagnostic modalities as well as its pathophysiology and the possible inclusion within the IgG4-related disease (IgG4-RD) spectrum. Finally, a diagnostic–therapeutic algorithm for a standardized approach to iRPF is proposed.

Methods

The PubMed Internet database was searched. Articles were selected based on the relevance of abstract, article type and impact of the journal.

Results

iRPF and IgG4-RD share a common autoimmune aetiology. Diagnostics are multimodal and based on imaging. Ruling out malignancy should be of primary concern. Complications are mostly of renal or vascular origin due to compression of retroperitoneal structures. Corticosteroids remain the first-line treatment regimen and are mostly successful, but evidence supporting alternative immunosuppressive and anti-inflammatory treatments is growing. Long-term therapy, as well as follow-up, is paramount in this chronic and often relapsing disease.

KEYWORDS:

• Idiopathic retroperitoneal fibrosis
• algorithm
• IgG4-related disease
• autoimmune
• peri-aortitis

Biographical note

Eduard Roussel: Resident in Urology and part-time PhD student associated with the Catholic University of Leuven, Belgium. ORCID ID: 0000-0002-7835-5536.

Jasper Callemeyn: Resident in Internal Medicine and full-time PhD student associated with the Catholic University of Leuven, Belgium. ORCID ID: 0000-0003-2235-9842.

Wouter Van Moerkercke: Gastroenterologist at AZ Groeninge, Kortrijk, Belgium, and associated with the Catholic University of Leuven, Belgium. Member of the Belgian Inflammatory Bowel Disease Research and Development Group (BIRD).

Disclosure statement

No potential conflict of interest was reported by the authors.