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.

Current approach to diagnosis and management of retroperitoneal fibrosis

“…In contrast, ureterolysis, an aggressive surgical treatment, is carried out to relieve ureteral obstruction and salvage renal function in selected patients, but the optimal indication for it is controversial. 4,6,[9][10][11] In the present review, we summarize the current concept of RPF, and discuss the optimal strategy for diagnosis and management.…”

“…Because of the good responsiveness to medical therapy, ureteral obstruction is usually managed conservatively with ureteral stenting or PNS. In contrast, ureterolysis, an aggressive surgical treatment, is carried out to relieve ureteral obstruction and salvage renal function in selected patients, but the optimal indication for it is controversial 4,6,9–11 …”

Simultaneous occurrence of pancreatic mixed acinar-ductal adenocarcinoma and primary follicular lymphoma of the duodenum, accompanied by increased number of IgG4 plasma cells in tumor-free parenchyma as concomitant IgG4-related disease or reaction to tumor? A case report

“…AIP type I is included in the spectrum of IgG4-related disease. The diagnosis of this fibroinflammatory disease with a heterogeneous clinical picture is based on the clinical presentation and typical histological changes, such as lymphoplasmacytic infiltrate, fibrosis (at least focally arranged in a storiform pattern), obliterative phlebitis and an elevated number of IgG4 plasma cells which is defined by the Consensus statement (30 cells per high power field for pancreas biopsy and 50 for resections; the proposed IgG4-to-IgG ratio is more than 40%) [14,15,16,17,18]. IgG4 related disease can affect different organs and usually manifests as a tumorous mass in the affected organ [19].…”

Włóknienie zaotrzewnowe jako rzadka choroba układowa przysparzająca wielu trudności diagnostycznych — opis przypadku i przegląd piśmiennictwa

“…Daje ono szansę szybkiego osiągnięcia remisji -duża część zmian radiologicznych cofa się już w czasie pierwszego tygodnia terapii. Większość pacjentów zgłasza poprawę już w ciągu pierwszych 2 tygodni leczenia [30]. W badaniach laboratoryjnych obserwuje się normalizację parametrów stanu zapalnego.…”