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Comprehensive assessment and outcomes of patients with chronic periaortitis

Gozde Kubra Yardimci1, Selin Ardali Düzgün2, Bayram Farisogullari1

  • 1Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, and Hacettepe Universitiy Vasculitis Research Center (HUVAC), Hacettepe University, Ankara, Turkey.

Clinical and Experimental Rheumatology
|March 29, 2022
PubMed

Insights

Chronic periaortitis (CP) is a fibro-inflammatory disorder with limited data. This study identified CP features and outcomes, finding idiopathic retroperitoneal fibrosis most common and favorable treatment responses to steroids with cyclophosphamide or rituximab.

Area of Science:

  • Vascular inflammation
  • Fibro-inflammatory disorders
  • Rare diseases

Background:

  • Chronic periaortitis (CP) is an underdiagnosed fibro-inflammatory condition.
  • Limited data exists on CP follow-up and outcomes.
  • This study addresses the knowledge gap in CP characteristics and prognosis.

Purpose of the Study:

  • To identify clinicopathologic, laboratory, and radiologic features of CP.
  • To evaluate the outcomes of CP patients.
  • To establish optimal follow-up imaging intervals.

Main Methods:

  • Retrospective review of medical records from the HUVAC database.
  • Inclusion of patients diagnosed with CP based on imaging, histopathology, and clinical findings.
  • Analysis of demographics, laboratory results, clinical presentation, and imaging data.

Main Results:

  • 51 patients (37 male/14 female, median age 63) were included with a median follow-up of 40 months.
  • Idiopathic retroperitoneal fibrosis was the most common presentation (82%), with 32 patients having IgG4-related CP.
  • Thoracic involvement (15.6%) and venous involvement (31.6%) were observed; steroids with cyclophosphamide (CYC) or rituximab (RTX) showed favorable outcomes with 64.1% regression on follow-up imaging at 6-9 months.

Conclusions:

  • Idiopathic retroperitoneal fibrosis is the most frequent CP presentation.
  • Thoracic and venous involvement are not uncommon in CP.
  • Steroids combined with CYC/RTX are effective treatments, with optimal follow-up imaging at 6-9 months.
Abstract

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