eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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5/2016
vol. 54
 
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abstract:
Review paper

Retroperitoneal fibrosis – the state-of-the-art

Marta Runowska
,
Dominik Majewski
,
Mariusz Puszczewicz

Reumatologia 2016; 54, 5: 256-263
Online publish date: 2016/11/28
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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.
keywords:

retroperitoneal fibrosis, Ormond’s disease, IgG4-related disease, chronic periaortitis



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