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IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening

Ichiro Mizushima1,2, Satomi Kasashima3, Yasunari Fujinaga4

  • 1a Division of Rheumatology , Kanazawa University Hospital , Kanazawa , Japan.

Modern Rheumatology
|November 27, 2018
PubMed

Insights

IgG4-related disease can affect blood vessels, causing periaortitis and periarteritis. Understanding diagnosis and treatment for this IgG4-related vasculitis is crucial for better patient outcomes.

Area of Science:

  • Vascular Medicine
  • Immunology
  • Rheumatology

Background:

  • Periaortitis and periarteritis are recognized clinical manifestations of IgG4-related disease (IgG4-RD).
  • Vascular involvement occurs in 10-30% of IgG4-RD cases and can present as isolated lesions.
  • IgG4-related vasculitis, similar to other large vessel vasculitides, can lead to severe clinical complications.

Purpose of the Study:

  • To review the clinical and pathological features of IgG4-related periaortitis and periarteritis.
  • To discuss challenges in diagnosing and treating IgG4-related vasculitis.
  • To identify key areas for future research to improve understanding of IgG4-related vascular disease.

Main Methods:

  • Literature review of clinical and pathological studies on IgG4-related periaortitis/periarteritis.
  • Analysis of diagnostic criteria used in reported cases.
  • Discussion of therapeutic strategies and unresolved issues in IgG4-related vasculitis.

Main Results:

  • Inconsistent diagnostic criteria hinder accurate assessment of IgG4-related periaortitis/periarteritis frequency.
  • Optimal therapeutic strategies for IgG4-related vasculitis remain undetermined.
  • Significant clinical consequences can arise from IgG4-related large vessel vasculitis.

Conclusions:

  • Standardized diagnostic criteria are needed for IgG4-related periaortitis and periarteritis.
  • Further research is required to establish optimal treatment protocols for IgG4-related vasculitis.
  • Clarifying diagnostic and therapeutic approaches will enhance management of IgG4-related vascular disease.

Keywords:
IgG4-related diseaseorgan-specific diagnostic criteriaperiaortitisperiarteritis

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