Autoinflammatory & rare systemic diseases

Reduced follicular helper T cells in peripheral blood marks a distinct feature of retroperitoneal fibrosis

Rheumatology (Oxford)Published Mechanistic study

Authors' conclusion

Our findings suggest that cTFH cells in the circulating blood of RPF do not expand but may undergo chemotactic migration to fibrotic tissues, marking a distinct feature of RPF and may serve as a potential biomarker.

Study context

Population
Patients with idiopathic retroperitoneal fibrosis (RPF)
Participants
23 patients
Outcome
Changes of T and B lymphocyte subsets in peripheral blood and their associations with disease activity and severity
Methodological detailsprospective follow-up for 48 weeks, flow cytometry, RNA sequencing following CD4+ T cell sorting
  • Prospective follow-up for 48 weeks
  • Flow cytometry
  • RNA sequencing following CD4+ T cell sorting

Main finding

As reported in the abstract

Circulating follicular helper T (cTFH) cells
  • cTFH cells significantly reduced in RPF patients
  • cTFH cells positively correlated with disease severity and TFH expansion in fibrotic tissues
  • cTFH cells further declined upon disease amelioration
  • Unchanged proliferation and low cytokine secretion in cTFH cells
  • CXCL13 expression markedly elevated in CD4+ T cells from both fibrotic tissues and peripheral blood
  • IL-21, IL-6 and Bcl elevated only in fibrotic tissues

Original publication

Reduced follicular helper T cells in peripheral blood marks a distinct feature of retroperitoneal fibrosis. Rheumatology (Oxford). Published 25 May 2026. doi:10.1093/rheumatology/keag274

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