Retroperitoneal Fibrosis: Review of modern literature

Marios Stavropoulos, Athanasios Papatsoris

Abstract


Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of an extensive inflammatory fibrotic mass in the retroperitoneum. The aorta, inferior vena cava (IVC) and iliac vessels are usually involved. Ureteral obstruction is observed in most of the cases. Recently, RPF is reported to belong to IgG4-related diseases, which is proposed as a new clinical entity. Imaging studies play a primary role in the diagnosis of RPF, with the CT scan being the modality of choice. Glucocorticoids are the mainstay of treatment, used either as monotherapy or in combination with other agents. If conservative measures fail, surgical intervention is necessary. Recently, minimally invasive techniques have been applied.


H οπισθοπεριτοναϊκή ίνωση είναι μια σπάνια ασθένεια που χαρακτηρίζεται από την παρουσία μιας εκτεταμένης, φλεγμονώδης, ινώδης μάζας στο οπίσθιο περιτόναιο, που συνήθως περιβάλλει την αορτή, την κάτω κοίλη φλέβα και τα λαγόνια αγγεία.Στις περισσότερες περιπτώσεις παρατηρείται συνήθως και απόφραξη των ουρητήρων. Πρόσφατα, αναφέρθηκε πως η οπισθοπεριτοναϊκή ίνωση ανήκει στις ασθένειες σχετιζόμενες με IgG4, που αποτελούν μια νέα κλινική οντότητα. Οι απεικονιστικές μέθοδοι παίζουν πρωταρχικό ρόλο στη διάγνωση της οπισθοπεριτοναϊκής ίνωσης, με την αξονική τομογραφία να αποτελεί την εξέταση εκλογής.
Τα γλυκοκορτικοειδή αποτελούν την κυριότερη θεραπεία και χρησιμοποιούνται είτε ως μονοθεραπεία είτε σε συνδυασμό με άλλους παράγοντες. Εάν οι συντηρητικές μέθοδοι αποτύχουν, συνήθως απαιτείται χειρουργική παρέμβαση. Πρόσφατα, ελάχιστα επεμβατικές μέθοδοι έχουν εφαρμοσθεί.

H οπισθοπεριτοναϊκή ίνωση είναι μια σπάνια ασθένεια που χαρακτηρίζεται από την παρουσία μιας εκτεταμένης, φλεγμονώδης, ινώδης μάζας στο οπίσθιο περιτόναιο, που συνήθως περιβάλλει την αορτή, την κάτω κοίλη φλέβα και τα λαγόνια αγγεία.

Στις περισσότερες περιπτώσεις παρατηρείται συνήθως και απόφραξη των ουρητήρων. Πρόσφατα, αναφέρθηκε πως η οπισθοπεριτοναϊκή ίνωση ανήκει στις ασθένειες σχετιζόμενες με IgG4, που αποτελούν μια νέα κλινική οντότητα. Οι απεικονιστικές μέθοδοι παίζουν πρωταρχικό ρόλο στη διάγνωση της οπισθοπεριτοναϊκής ίνωσης, με την αξονική τομογραφία να αποτελεί την εξέταση εκλογής.

Τα γλυκοκορτικοειδή αποτελούν την κυριότερη θεραπεία και χρησιμοποιούνται είτε ως μονοθεραπεία είτε σε συνδυασμό με άλλους παράγοντες. Εάν οι συντηρητικές μέθοδοι αποτύχουν, συνήθως απαιτείται χειρουργική παρέμβαση. Πρόσφατα, ελάχιστα επεμβατικές μέθοδοι έχουν εφαρμοσθεί.


Keywords


Retroperitoneal fibrosis; Ormond’s disease; IgG4; glucocorticoids;Oπισθοπεριτοναϊκή ίνωση; νόσος Ormond; IgG4; γλυκοκορτικοειδή

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DOI: http://dx.doi.org/10.19264/hj.v27i2.78