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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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Autoinflammatory associated vasculitis.

Shira Ginsberg1, Itzhak Rosner1, Michel Rozenbaum1

  • 1Rheumatology Unit, Bnai-Zion Medical Center, 47 Eliyahu Golomb St, Haifa, 33048 Israel.

Seminars in Arthritis and Rheumatism
|September 11, 2016
PubMed
Summary
This summary is machine-generated.

Autoinflammatory diseases, often linked to innate immunity defects, can present with vasculitis. This study introduces "autoinflammatory associated vasculitis" and urges its consideration in vasculitis diagnoses.

Keywords:
Autoinflammatory diseasesLeukocytoclastic vasculitis

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Area of Science:

  • Immunology
  • Rheumatology
  • Dermatology

Background:

  • Autoinflammatory diseases (AIDs) involve recurrent inflammation due to innate immunity defects.
  • Skin manifestations are common in AIDs, but vasculitis is not a recognized feature.
  • Current nomenclature does not list AIDs as a cause of systemic vasculitis.

Observation:

  • Three patients with distinct AIDs presented with leukocytoclastic vasculitis as a primary symptom.
  • Literature review confirms vasculitis occurs within the spectrum of AIDs.
  • A pathophysiology for AIDs-associated vasculitis is proposed.

Findings:

  • Leukocytoclastic vasculitis can be a major presenting symptom of AIDs.
  • The term "autoinflammatory associated vasculitis" is proposed.
  • AIDs should be included in the differential diagnosis for vasculitis.

Implications:

  • This work expands the understanding of AIDs manifestations.
  • It highlights the need to consider AIDs in vasculitis patients.
  • The proposed term "autoinflammatory associated vasculitis" aids in classification and research.