Association between large vessel vasculitis and inflammatory bowel disease: a case-control study

Objectives: To describe the characteristics and outcome of patients with the association of large vessel vasculitis (LVV, Takayasu arteritis [TA] or giant cell arteritis [GCA]) and inflammatory bowel disease (IBD). Methods: An observational, multicentre, retrospective case-control study. Cases were LVV-IBD patients from European countries, whereas controls had isolated LVV (iLVV). Results: 39 TA-IBD and 12 GCA-IBD cases were enrolled, compared with 52 isolated GCA (iGCA) and 93 isolated TA (iTA) controls. LVV occurred after IBD in 56% in TA-IBD and 75% in GCA-IBD, with a median interval of 1 year (IQR 1-7) in TA-IBD and 8.6 years (IQR 1-17.7) in GCA-IBD. Crohn's disease was more common in TA-IBD (67%), whereas ulcerative colitis was more common in GCA-IBD (58%). Compared with iTA, TA-IBD were significantly younger at diagnosis of TA (median age 27 vs 37 years, p< 0.001) and had more upper limb claudication (36% vs 12%, p= 0.006). GCA-IBD patients had more frequent arterial thickening or stenosis than controls (75% vs 30%, respectively, p= 0.044), and tended to more frequently involve gastrointestinal arteries (20% vs 0%, respectively, p= 0.06). LVV occurred in IBD patients despite treatment with glucocorticoids (36%), azathioprine (25%), or TNF-alpha blockers (29%). The presence of the IBD was not associated with a higher LVV relapse rate in multivariate analysis (adjusted hazard ratio [aHR] 0.62 [0.13-2.83] for GCA and aHR 0.92 [0.44-1.89] for TA). Conclusion: This study identifies specific clinical and imaging characteristics of LVV-IBD patients, in particular a more severe vascular presentation of GCA-IBD patients compared with iGCA patients.