Background: In the last decade, we have come to better understand and manage the vasculitides.
The classification of vasculitides has been revised. Genome- wide association studies and
linkage analyses have been undertaken in hope of better understanding the pathogenesis of vasculitides.
Comprehensive genetic studies have highlighted new pathways that may guide us in more
targeted therapies. Description of the monogenic forms of vasculitis, such as deficiency of adenosine
deaminase type 2 (DADA2), Haploinsufficiency of A20 (HA20), have introduced a new perspective
to vasculopathies, and introduced alternative treatments for these diseases.
Conclusion: In this review, the important discoveries in pathogenesis and consensus treatment recommendations
from the past decade will be summarized.
Keywords: Systemic vasculitis, treatment, pathogenesis, DADA2, genetic studies, pathways.
Rights & PermissionsPrintExport