Vascular Damage In Primary Systemic Vacsulitis: Current Knowledge And Future Perspectives

Primary systemic vasculitides (PSV) constitute a heterogeneous group of rare, chronic and often recurrent systemic autoimmune/autoinflammatory diseases, that are classified according to the size of the predominantly affected vessels into large (LVV), medium (MVV) and small vessel vasculitis (SVV). These disorders are characterized by multi-level heterogeneity that spans clinical phenotype, histologic patterns, pathogenetic mechanisms and treatment selection strategies. In PSV, the hallmark vascular lesion is transmural inflammatory degeneration of the vessel wall, arising from several pathogenetic mechanisms, including T cell-mediated immunity, immune complex formation and the action of anti-neutrophil cytoplasmic antibodies. The introduction of combined therapeutic interventions involving corticosteroids, conventional immunosuppressives and contemporary biologics (monoclonal antibodies targeting key cytokines and effector cells) has transformed PSV from almost invariably lethal diseases into ones with markedly improved outcomes.



Despite considerable progress in systemic vasculitis research over the past decade, several unmet needs still hinder the optimal care of these patients and involve:

a) Reliable biomarkers for the accurate assessment of disease activity and cumulative vascular damage at disease diagnosis and follow-up,

b) Robust predictors of disease relapse that persist despite apparently adequate immunosuppressive treatment that will enable tailored therapy, fewer recurrences and reduce drug-related side effects,

c) Early indicators of long-term cardiovascular complications, particularly ischemic events and aneurysm formation that drive the excess mortality in these disorders, and

d) Evidence-based guidance on biologic therapies that would specify when to initiate treatment in active disease and when to withdraw it, once remission is achieved.

The fulfillment of these critical needs demands a deeper understanding of the pathogenetic pathways that cause vascular inflammation and damage. This special issue therefore seeks original research and state-of-the-art reviews that address these unmet needs.



We welcome the submission of Original Research, Review, Mini Review, and Perspective articles on themes including, but not limited to:

• Pathogenesis of large and medium vessel vasculitis

• Identification of new clinical and/or histopathological phenotypes of primary systemic vasculitides

• Biomarkers of disease activity, monitoring and response to treatment in large and medium vessel vasculitis

• Predictors of CVD complications in large and medium vessel vasculitis

• Clinical trials for large, medium and small vessel vasculitis

• All the above topics using as disease model ANCA-associated vasculitides, IgG4 aortitis, cryoglobulinemic vasculitis and Behcet’s disease with vascular involvement