Systemic Vasculitis: Advancement in Pathogenesis, Clinical Features, Management and Therapeutic Strategies

Dear Colleagues,

Immune-mediated Systemic Vasculitis (ISV) is a heterogeneous group of diseases involving blood vessel inflammation. Usually, these diseases are classified according to the size of the involved vessels into small, medium, or large vessel vasculitis.

Most are rare diseases, but often with severe life-threatening manifestations and multi-organ involvement. They can affect anyone, though some types are more common among certain age groups.

Management and classification of ISV are sometimes difficult due to unknown etiology and heterogeneity in clinical presentation. Furthermore, the management of both small and large vessel vasculitis is challenging because of a lack of robust markers of disease activity and the frequent involvement of multiple organs, often leading to the need for a multidisciplinary approach.

Recently, there have been several notable advances in ISV research, including developing new classification criteria, new therapeutic tools, and general advances in pathophysiology and associated risk factors. Treatment regimens continue to improve with the use of different immunosuppressive medications and newer therapeutic approaches, such as biologic agents, along with discoveries and advances in our understanding of ISV's genetic and etiopathological basis.

There is, however, a need for a standardized approach to the management of these rare rheumatic diseases.

The heterogeneity of ISV and its rareness make inexact incidence and prevalence estimates. Moreover, much more investigation is needed on the causes and mechanisms affecting the development and progression of these disorders, while more studies are needed to discover innovative treatments, their combinations, and new shared therapeutic procedures.

Therefore, improving the knowledge of ISV, from basic science to clinical practice, is critical.

Challenges in studying systemic vasculitis lie in achieving accurate epidemiological data and making efforts to obtain significant progress in terms of etiological mechanism, clinical behavior, genetic/epigenetic basis of the diseases, as well as early diagnosis, treatment, and management of patients.

In the near future, these advances will allow more precise knowledge of these diseases, enabling clinicians to offer more targeted and individualized patient management.

Dr. Giulia Cassone
Guest Editor

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• immune-mediated systemic vasculitis
• etiology
• epidemiology
• therapy
• diagnosis
• classification
• risk factors
• genetics
• management