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

1, 2, 3, 4, 5, 6, 7, 8, 9

  1. Department of Internal Medicine and Clinical Immunology, CHRU Lille; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
  2. Department of Pathology, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France; Sorbonne Universités, Université Pierre et Marie Curie, Paris, France; INSERM, UMR S 1155, Paris, France.
  3. Department of Vascular Medicine, Hôpital Européen Georges-Pompidou, Assistance Publique Hôpitaux de Paris, APHP, Centre National de Référence Maladies Vasculaires Rares, Paris Descartes University, Sorbonne Paris Cite, PARCC, INSERM U970, Paris, France.
  4. Inserm, U1189-ONCO-THAI, CHRU Lille, University Lille Nord de France; Department of Radiology, CHU Lille, Université de Lille, France.
  5. Department of Pathology, Centre de Biologie Pathologie, CHRU Lille, University Lille Nord de France; INSERM, UMR S 995, Lille, France.
  6. Department of Internal Medicine and Clinical Immunology, CHRU Lille; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
  7. Department of Internal Medicine and Clinical Immunology, CHRU Lille; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
  8. Department of Internal Medicine and Clinical Immunology, CHRU Lille; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
  9. Dept. of Internal Medicine and Clinical Immunology, CHRU Lille; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares; FHU Immune-Mediated Inflammatory Diseases & Targeted Therapies (IMMINeNT), Lille, France. marc.lambert@chru-lille.fr

CER10595 Submission on line
2018 Vol.36, N°3 - PI 0371, PF 0375
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Rheumatology Article

 

Abstract

OBJECTIVES:
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.
METHODS:
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.
RESULTS:
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.
CONCLUSIONS:
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.

PMID: 29465374 [PubMed]

Received: 22/05/2017 - Accepted : 27/06/2017 - In Press: 14/02/2018 - Published: 17/05/2018