Autoinflammatory Disease-Associated Vasculitis/Vasculopathy
Purpose of Review
Autoinflammatory diseases (AIDs) constitute several disorders that share similar characteristics, clinical features, disease course, and prognosis. They are characterized by the presence of recurrent episodes of unprovoked inflammation due to dysregulated innate immune system in the absence of autoantibodies or infections. AIDs include periodic fever syndromes and other less commonly growing list of syndromes. In this review, vasculitis associated with different AIDs will be highlighted.
Vasculitis is inflammation and necrosis of the blood vessels causing impaired blood flow, ischemia, and infarction of the dependent tissues. It is a very rare manifestation of AIDs and when it occurs, the skin is the most affected tissue than any other organs such as kidneys, lungs, or CNS.
Although vasculitis and AIDs share similar manifestations such as fever, skin rashes, and neuropathy, vasculitis is not a characteristic feature of AIDs and still not clear if it represents a main clinical feature or a manifestation of other disease process.
KeywordsAutoinflammatory diseases Periodic fever syndrome Vasculitis Leukocytoclastic vasculitis Familial Mediterranean fever Blau syndrome SAVI DADA2 HA20
The author acknowledges Professor Luis R. Espinoza, who reviewed this paper.
Compliance with Ethical Standards
Conflict of Interest
The author declares that there is no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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