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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.
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 …
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.
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