title: Retroperitoneal fibrosis - the state-of-the-art.
abstract: Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed.
authors.affiliation: Department of Rheumatology and Internal Diseases, Poznan University of Medical Science, Poland.
authors.forename: Marta
authors.initials: M
authors.lastname: Runowska
authors.affiliation: Department of Rheumatology and Internal Diseases, Poznan University of Medical Science, Poland.
authors.forename: Dominik
authors.initials: D
authors.lastname: Majewski
authors.affiliation: Department of Rheumatology and Internal Diseases, Poznan University of Medical Science, Poland.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Puszczewicz
keywords: IgG4-related disease; Ormond’s disease; chronic periaortitis; retroperitoneal fibrosis