Article Text

Download PDFPDF
Rituximab for chronic periaortitis without evidence of IgG4-related disease: a long-term follow-up study of 20 patients
  1. Maria Letizia Urban1,
  2. Federica Maritati1,
  3. Alessandra Palmisano1,
  4. Paride Fenaroli2,
  5. Francesco Peyronel2,
  6. Giorgio Trivioli3,
  7. Stefania Ferretti1,
  8. Consuelo De Biase4,
  9. Peter C Grayson5,
  10. Francesco Pegoraro6,
  11. Domenico Prisco7,
  12. Paola Romagnani8,
  13. Giacomo Emmi1,
  14. Augusto Vaglio9
  1. 1 Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy
  2. 2 Nephrology, Parma University Hospital, Parma, Italy, Italy
  3. 3 Nephrology Unit, Department of Clinical and Experimental Medicine, University of Parma, Parma, Italy
  4. 4 Nephrology, University of Turin, Torino, Italy
  5. 5 Pediatrics, National Institutes of Health/NIAMS, Bethesda, Maryland, USA
  6. 6 Azienda Ospedaliero Universitaria Ospedale Pediatrico Meyer, Firenze, Italy
  7. 7 Department of Experimental and Clinical Medicine, University of Florence, AOU Careggi, Florence, Italy
  8. 8 Nephrology and Dialysis Unit, Meyer Children’s University Hospital, Florence, Italy
  9. 9 Biomedical Experimental and Clinical Sciences, University of Florence, Firenze, Italy
  1. Correspondence to Dr Augusto Vaglio, University of Florence, 50121 Firenze, Italy; augusto.vaglio{at}

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Chronic periaortitis (CP) is a rare condition characterised by a peri-aortoiliac fibro-inflammatory tissue. A total of 20%–50% of the cases are immunoglobulin G4 (IgG4)-related, based on histological evidence of IgG4+ plasma cell infiltration (on a background of dense lymphoplasmacytic infiltrates, storiform fibrosis and tissue eosinophilia) and/or increased serum IgG4.1

Glucocorticoids are the first-line therapy for CP.2 However, some patients are refractory, frequently relapsing or have contraindications to glucocorticoids. The anti-CD20 monoclonal antibody rituximab proved efficacious in systemic forms of IgG4-related disease (IgG4-RD) including IgG4-related CP,3 but data on IgG4-unrelated CP are scarce.4–6 In this study, we tested rituximab in CP patients without evidence of IgG4-RD who had relapsing/refractory disease or contraindications to standard-dose glucocorticoids.

We included patients with active, IgG4-unrelated CP who received rituximab (October 2009 to April 2017). Online supplementary methods describe the diagnostic and follow-up procedures, the definitions of remission and refractory, and the statistical analysis.

Supplemental material


Twenty consecutive patients were included. Two of them were previously reported.5 Of the eight patients with available CP biopsies, none had significant IgG4+-plasma cell infiltration. None had other biopsy-proven …

View Full Text


  • Handling editor Josef S Smolen

  • Contributors MLU, FM and AV designed the study, analysed the data and drafted the manuscript. MLU, FM, AP, PF, F Peyronel, GT, SF, CDB, PCG, GE and AV followed the patients and collected the data. F Pegoraro and MLU generated the figures. PCG, DP, PR and GE critically reviewed the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement statement Given the retrospective nature of this work, it was not possible to involve patients or the public in the design, or conduct, or reporting, or dissemination of this research.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.