Vasculitis is characterized by inflammation and destruction of blood vessels, resulting in downstream ischemic tissue damage. Diagnosis of vasculitis is a careful exercise in clinical-pathologic correlation, depending upon the clinical manifestations, organs involved, the size of affected blood vessels, imaging, and laboratory findings. While some vasculitis subtypes may be confined to the skin, serious internal organ involvement or underlying disease states may also occur. Accordingly, the skin plays an important role in the diagnostic process and may be prognostically important in some cases, signifying more severe systemic disease. The skin also provides opportunities for tissue-based translational research, improving understanding of disease pathophysiology. Dermatologists, therefore, play a critical role in evaluating vasculitis and helping to advance vasculitis clinical care and research. Recent updates in vasculitis nomenclature and terminology, evidence-based diagnosis, pathogenesis, and investigations of targeted therapies are changing vasculitis research and leading to fundamental shifts in disease management. Treatment advances favoring evidence-based and targeted, rather than broadly immunosuppressive, therapies are in development, while a multicenter trial for skin-limited vasculitis is ongoing. Collaborative multidisciplinary research networks are key to current and future advances in vasculitis research. In this review, we describe recent developments in vasculitis clinical care and research, starting with a discussion of efforts to develop diagnostic and classification criteria, followed by updates on the evaluation and treatment of vasculitis.
Keywords: NETosis; Vasculitis; cutaneous vasculitis; nomenclature; targeted therapy.
2021 Annals of Translational Medicine. All rights reserved.