Systemic Vasculitis: Rare Pathologies with Complex Diagnosis That Look to Car-T

Systemic vasculitides are a heterogeneous group of rare autoimmune diseases characterized by persistent inflammation of the blood vessels, potentially affecting different organs such as the heart, kidneys, lungs and brain. If not recognized and treated promptly, these conditions can lead to severe complications even in young individuals with no known risk factors.

The clinical manifestations of vasculitis vary according to the type and size of the vessels involved and can range from nonspecific symptoms such as fever and weight loss to very typical manifestations.

Among the main forms of systemic vasculitis, we find those that affect small blood vessels, including eosinophilic granulomatosis with polyangiitis, characterized by high levels of certain white blood cells (eosinophils) in the blood and tissues, often associated with asthma and rhinosinusitis, but which can also involve the heart and nervous system, or microscopic polyangiitis and granulomatosis with polyangiitis, which mainly affect the kidneys, skin and lungs. Other important forms of vasculitis that affect larger blood vessels are giant cell arteritis (also known as Horton's arteritis), the most common form of vasculitis today, which occurs mainly in people over 60 years of age, and Behçet's syndrome, a condition that involves inflammation of vessels of varying sizes, characterized among other things by recurrent inflammatory thrombotic episodes. More recently, genetically determined forms of vasculitis have been described, also characterized by various manifestations, such as recurrent thrombotic ones, including stroke, heart attack and pulmonary embolism.

Given the variability of the clinical presentation of these rare conditions, the approach to these pathologies is multidisciplinary and the diagnosis is often complex, with a diagnostic delay still common despite the great progress made in recent years by the international scientific community and in particular by the European Vasculitis Society Euvas.

The guidelines of the European Alliance of Associations for Rheumatology (Eular) recommend different therapeutic approaches for the management of systemic vasculitis, which still today include the use of systemic cortisone, which we combine with both traditional immunosuppressive drugs such as cyclophosphamide, but increasingly with new molecules capable of reducing inflammation in a targeted and selective way. Recently, the AIFA approved the reimbursement of avacopan, an oral inhibitor of the complement receptor C5a, indicated for the treatment of granulomatosis with polyangiitis and microscopic polyangiitis. The monoclonal antibodies mepolizumab and benralizumab, capable of blocking the survival of eosinophils, are radically changing the treatment and quality of life of patients affected by eosinophilic granulomatosis with polyangiitis. Finally, drugs such as JAK inhibitors, already in use for the treatment of rheumatoid arthritis, are demonstrating efficacy and safety in the treatment of systemic vasculitis such as giant cell arteritis.