Takayasu arteritis: Prevalence and clinical presentation in Switzerland

PLoS One. 2021 Jun 18;16(6):e0250025. doi: 10.1371/journal.pone.0250025. eCollection 2021.

Abstract

Objective: Takayasu arteritis (TAK) is a rare immune-mediated vasculitis of the aorta and its branches. Aims were to calculate prevalence and incidence in Switzerland, to assess disease activity and performance of MR-Angiography (MRA).

Methods: 31 patients were recorded in a database, 27 were followed prospectively up to 3 years. Prevalence was calculated based on data of the national statistical bureau. Disease activity was defined using the revised EULAR criteria. MRA depicted stenotic changes and aortic wall enhancement.

Results: A disease prevalence of 14.5/1.000.000 inhabitants and an incidence of 0.3/1.000.000 per year was calculated. Aortic wall enhancement was found in 10 patients while in clinical and serological remission. EULAR criteria missed 5 patients with disease activity with isolated elevations of ESR/CRP. Arterial stenosis did not change over time in 5 cases, it improved in 2 and increased in 7. At follow-up 16 patients were treated with tocilizumab, 11/16 in monotherapy, 5 patients were treatment-free, 25/27 stayed in remission.

Conclusion: In addition to prevalence and incidence, our data show that MRA qualifies to detect subclinical disease activity, but, on the other hand, that EULAR criteria may miss disease activity in case of isolated elevation of ESR/CRP.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Angiography / methods*
  • Male
  • Middle Aged
  • Prevalence
  • Prognosis
  • Prospective Studies
  • Switzerland / epidemiology
  • Takayasu Arteritis / diagnostic imaging
  • Takayasu Arteritis / epidemiology*
  • Takayasu Arteritis / pathology*
  • Tomography, X-Ray Computed / methods*
  • Young Adult

Grants and funding

The study was funded by the Research Funds of the Division of Rheumatology, Department of Rheumatology, Immunology and Allergology, University Hospital of Bern, Switzerland. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.