Takayasu arteritis in the context of polyautoimmunity: clinical clustering, shared immunopathogenesis, and cardiovascular phenotypes

Abstract Background Takayasu arteritis is a rare, chronic large-vessel vasculitis that primarily affects young women, particularly the aorta and its branches. It is traditionally regarded as an isolated vascular disease, and its coexistence with other systemic autoimmune diseases is uncommon, having been described mainly in case reports and small series. These reports, together with shared genetic and immunological features, have nevertheless prompted interest in whether TAK can be considered within the concept of polyautoimmunity. Objective This narrative review explores the relationship between Takayasu arteritis and polyautoimmunity, highlighting current evidence on shared immune mechanisms, disease clustering, and cardiovascular manifestations, and considers whether these findings support an autoimmune spectrum. Methods A comprehensive literature search was conducted in PubMed, Google Scholar, and the Cochrane Library for studies published since 2002. MeSH terms and keywords related to Takayasu arteritis (TAK), polyautoimmunity, and cardiovascular manifestations were used. Evidence from international registries, meta-analyses, and retrospective cohorts was reviewed. Studies were selected based on relevance to shared immunopathogenic mechanisms, the 2022 ACR/EULAR classification criteria, and Numano angiographic types. The literature was synthesized to evaluate the impact of coexisting autoimmune diseases on cardiovascular outcomes and treatment response in TAK. Results Takayasu arteritis (TAK) has been reported to coexist with other autoimmune diseases, particularly spondyloarthritis-spectrum disorders, and, less frequently and largely in case reports, systemic lupus erythematosus, rheumatoid arthritis, Behçet disease, and inflammatory bowel disease. These reported associations are supported by shared genetic susceptibility, especially HLA-B52, and common immune pathways involving the Th1/Th17 axis, IL-6, and TNF-α. Persistent activation of these pathways contributes to chronic systemic inflammation, which may worsen vascular damage and promote cardiovascular complications such as aortic regurgitation, renovascular hypertension, coronary ostial stenosis, and cerebrovascular ischemic events. Limited observational data suggest that coexisting autoimmune disease may be linked to a more treatment-resistant course, with a higher need for biologic agents such as tocilizumab or TNF inhibitors. Conclusion Although coexistence with other autoimmune diseases is uncommon and the supporting evidence is limited, considering Takayasu arteritis (TAK) within the spectrum of polyautoimmunity may help clinicians recognize shared immune mechanisms. Evaluation for coexisting autoimmune conditions at diagnosis and during follow-up, guided by clinical suspicion, may facilitate earlier recognition, guide individualized immunomodulatory treatment, and, where appropriate, enable a single therapeutic agent to address overlapping diseases. A multidisciplinary approach may further improve long-term outcomes by reducing the risk of severe cardiovascular and other end-organ complications.