Background/Purpose: Large vessel vasculitis (LVV) is the arteritis in aorta and its major branches, and classified into Takayasu arteritis (TAK) and giant cell arteritis (GCA). Some patients with aortic valve regurgitation (AR) or aortic aneurism need vascular surgery, and TAK patients sometimes manifest comorbidities including ulcerative colitis and other autoimmune diseases. However, their prevalence and clinical characteristics are poorly identified. The aim of this study is to determine the prevalence and clinical characteristics of patients who possessed complications or comorbidities of LVV.

Methods: 144 patients who were diagnosed as either TAK (n=132) or GCA (n=12) according to JCS or ACR classification criteria in Tohoku University during 2008-2017 were enrolled to this study. The prevalence and clinical characteristics of patients who received surgery or were complicated by other autoimmune disorders were retrospectively evaluated.

Results: Vascular surgeries were performed in 38 TAK patients (28.8 %) (Aortic valve replacement (AVR), 15; aortic root substitution, 8; other valve replacement, 4; coronary artery graft, 6; percutaneous transluminal angioplasty of renal artery, 5). The proportion of type V lesion was higher in patients who received surgery (43 %) compared to total population (31 %). Difference in vascular type was more evident in patients with AR and AVR. Type IIa accounted for 36 % of patients with AR and 52% of patients who received AVR, while it accounted 25 % in total population. 46.8 % of vascular surgeries were performed within a year after diagnosis (Figure 1), and the age was negatively correlated with C-reactive protein levels in such patients (p=0.008, r=0.35). These data revealed that there existed a group in which patients manifested rapidly progressive course. Surprisingly, autoimmune comorbidities were observed in 38.6 % of TAK patients and included inflammatory bowel diseases (IBD, 12 cases), thyroid diseases (9), hearing loss (5), rheumatoid arthritis (4), interstitial pneumonia (3), sternoclavicular joint arthritis (3), osteomyelitis (2), uveitis (2), and spondyloarthriopathy (SpA, 2). More than half of comorbidities were diseases in which tumor necrosis factor (TNF)- inhibitor was effective. Most of these comorbidities preceded TAK for about a few years.

Conclusion: 28.8 % of TAK patients received vascular surgeries, and half of them manifested rapidly progressive course. Particularly, type V lesion in young, type IIa lesion with AR, male, and complication of AR in the older are at high risk for vascular surgery. 38.6 % of TAK patients possessed autoimmune comorbidities, half of which can be complicated in SpA as well. This suggests similar pathomechanisms among TAK and SpA-related diseases and effectiveness of TNF inhibitors.

Figure 1. Years from diagnosis to surgery