Autoinflammatory Disease-Associated Vasculitis/Vasculopathy

Abstract

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.

Recent findings: 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.

Keywords: Autoinflammatory diseases; Blau syndrome; DADA2; Familial Mediterranean fever; HA20; Leukocytoclastic vasculitis; Periodic fever syndrome; SAVI; Vasculitis.