No specific imaging pattern can help differentiate IgG4-related disease from idiopathic retroperitoneal fibrosis: 18 histologically proven cases

Retroperitoneal fibrosis (RPF) is a rare disease, with unknown aetiology (idiopathic RPF: iRPF) in two-thirds of cases. A subset of iRPF may be a manifestation of IgG4-related disease (IgG4-RD). Thus, recognition of IgG4-RD-RPF is crucial to optimise patient's care with iRPF. The current study...

Full description

Saved in:
Bibliographic Details
Published in:Clinical and experimental rheumatology 2018-05, Vol.36 (3), p.371-375
Main Authors: Forestier, Alexandra, Buob, David, Mirault, Tristan, Puech, Philippe, Gnemmi, Viviane, Launay, David, Hachulla, Eric, Hatron, Pierre-Yves, Lambert, Marc
Format: Article
Language:English
Subjects:
Adult
Aged
Autoimmune Diseases
Biopsy
Case-Control Studies
Diagnosis, Differential
Female
Humans
Immunoglobulin G
Life Sciences
Magnetic Resonance Imaging
Male
Middle Aged
Retroperitoneal Fibrosis
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Retroperitoneal fibrosis (RPF) is a rare disease, with unknown aetiology (idiopathic RPF: iRPF) in two-thirds of cases. A subset of iRPF may be a manifestation of IgG4-related disease (IgG4-RD). Thus, recognition of IgG4-RD-RPF is crucial to optimise patient's care with iRPF. The current study aimed to examine imaging specific patterns, which could help differentiate between IgG4-RD-RPF and iRPF, and thus skip performing biopsies. This analysis included patients with iRPF and a retroperitoneal biopsy at the Lille University Hospital, France. We reviewed their baseline characteristics, clinical presentation, biological results and imaging features. Patients were classified in 3 groups according to histopathological characteristics of IgG4-RD as follows: highly suggestive of IgG4-RD, possible IgG4-RD, or non-evocative of IgG4-RD. Of the 18 patients analysed in the study, 4 (22%) patients had highly suggestive IgG4-RD-RPF, 8 (44%) possible IgG4-RD-RPF and 6 (33%) non-evocative IgG4-RD. We found no clinical, biological features nor specific imaging pattern that could help differentiate between the 3 groups. After ruling out all known causes of RPF, retroperitoneal biopsy is still necessary to ascertain the diagnosis of IgG4-RD-RPF. No specific pattern can be used to distinguish between IgG4-RD-RPF and iRFP.
ISSN:0392-856X