Aortitis: an update

Curr Opin Rheumatol. 2021 Jan;33(1):34-40. doi: 10.1097/BOR.0000000000000762.

Abstract

Purpose of review: Aortitis is the inflammation of the aorta due to various causes. Clinical presentations vary as well as the imaging findings. Exact pathogenetic mechanisms or triggering factors, as well as the best diagnostic and monitoring modalities and treatment strategies, are yet to be elucidated. We reviewed recent studies in aortitis and associated diseases.

Recent findings: Multiple cohort studies reporting long-term outcomes in patients with noninfectious aortitis were recently published. Comparative features of isolated aortitis were described. Six angiographic clusters for giant cell arteritis and Takayasu have been identified. New classification criteria have been proposed for IgG4-related disease by a data-driven method. The ultrasonographic slope sign and a halo score were described as specific imaging parameters in giant cell arteritis. The promising role of PET-computed tomography, not only in the diagnosis of aortitis but also in monitoring disease activity, has been noted. Results of in-vitro studies on Janus kinase (JAK)/signal transducers and activators of transcription and mammalian target of rapamycin (mTOR) pathways, comparative studies with leflunomide as an induction therapy, and a long-term follow-up study with tocilizumab may contribute to the management of Takayasu arteritis.

Summary: An impressive number of studies have addressed aortitis in recent years. However, there still is a lack of robust data on causes, monitoring disease activity by imaging and biomarkers, and drugs providing steroid-free remission in noninfectious aortitis.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Aorta / pathology
  • Aortitis / diagnosis*
  • Aortitis / drug therapy*
  • Aortitis / epidemiology
  • Female
  • Follow-Up Studies
  • Giant Cell Arteritis / diagnosis
  • Giant Cell Arteritis / epidemiology
  • Humans
  • Immunoglobulin G4-Related Disease / epidemiology
  • Immunosuppressive Agents / therapeutic use
  • Janus Kinases / metabolism
  • Leflunomide / therapeutic use
  • Male
  • Positron Emission Tomography Computed Tomography / methods
  • Takayasu Arteritis / diagnosis
  • Takayasu Arteritis / drug therapy
  • Takayasu Arteritis / epidemiology
  • Ultrasonography / methods

Substances

  • Antibodies, Monoclonal, Humanized
  • Immunosuppressive Agents
  • Janus Kinases
  • Leflunomide
  • tocilizumab