Challenges and Future Trends in Large Vessel Vasculitis

Abstract

Based on the Chapel Hill consensus criteria, the primary forms of vasculitis are classified by the predominant size of the affected blood vessels into large, medium, or small vessels. The two main forms of large vessel vasculitis (LVV) are giant cell arteritis and Takayasu arteritis, both of which are more prevalent in women while showing distinct geographic patterns of prevalence. The pathogenesis of LVV is complex and multifactorial, involving a combination of genetic predisposition, environmental and geographic triggers, immune dysregulation, and aging or (premature) senescence of the immune system and blood vessels. Diagnosis based on clinical presentation alone can be challenging because of the wide variety and often nonspecific symptoms that are associated with LVV. This has prompted the development of novel diagnostic tools to aid patient management, in particular advanced vascular imaging approaches. New therapies targeting specific immune pathways are now becoming available to improve outcomes while limiting the side effects associated with traditional glucocorticoid treatment. Advances in molecular imaging techniques may also enhance our ability to objectively monitor disease and treatment response in patients with LVV. Ongoing research aims to better understand the underlying mechanisms and to develop better targeted therapies for LVV, and to improve patient assessment and life time management. This narrative review will provide an in-depth update on current challenges and future trends in LVV, enhancing our understanding of its pathogenesis, diagnostic features, and management strategies, including disease- and treatment-related cardiovascular complications.