The Expanding Role of JAK Inhibitors in Vasculitis: From Large-Vessel Disease to Emerging Evidence in Monogenic Syndromes

Janus kinase (JAK) inhibitors have emerged as a potential therapeutic class across immune-mediated diseases, prompting interest in their application to vasculitis. Although data remain limited, early evidence suggests that JAK inhibition may offer clinical benefit in selected settings, including large-vessel vasculitis and Behçet’s disease and monogenic vasculopathies such as adenosine deaminase 2 deficiency (DADA2) and VEXAS syndrome, where treatment options are often restricted and disease control is challenging. In these conditions, modulation of intracellular signaling pathways may translate into improvements across systemic, cutaneous, and hematologic domains. Overall, while JAK inhibitors represent a promising and biologically plausible therapeutic avenue for difficult-to-treat vasculitic conditions, current evidence is derived almost entirely from retrospective studies and case series. Ongoing and future investigations are needed to define efficacy and optimal treatment strategies.