Large-vessel vasculitis diagnosed between 50 and 60 years: Case-control study based on 183 cases and 183 controls...

Age at onset of large-vessel vasculitis (LVV) is commonly used to distinguish giant cell arteritis (GCA) and Takayasu arteritis (TA). However, LVV between age 50 and 60 years may be difficult to classify.

We conducted a retrospective study including LVV aged between 50 and 60 years at onset (LVV_50-60, cases) and compared them to LVV aged over 60 years (LVV_>60, controls). LVV was defined histologically and/or morphologically. Controls fulfilled ACR 1990 criteria for GCA or presented isolated aortitis.

We included 183 LVV_50-60 and 183 gender-matched LVV_>60. LVV_50-60 had more frequent peripheral limb manifestations (23 vs. 5%), and less frequent cephalic (73 vs. 90%) and ocular signs (17 vs. 27%) than LVV_>60. Compared to LVV_>60, CT angiography and PET/CT scan were more frequently abnormal in LVV_50-60 (74 vs. 38%, and 90 vs. 72%, respectively), with aorta being more frequently involved (78 vs. 47%). By multivariate analysis, absence of cephalic symptoms, presence of peripheral limb ischemia and aorta involvement, and increased CRP level were significantly associated with LVV_50-60 presentation compared to LVV_>60. At last follow-up, compared to LVV_>60, LVV_50-60 received significantly more lines of treatment (2 vs. 1), more frequent biologics (12 vs. 3%), had more surgery (10 vs. 0%), and had higher prednisone dose (8.8 vs. 6.5 mg/d) at last follow-up, CONCLUSION: LVV onset between 50 and 60 years identifies a subset of patients with more frequent aorta and peripheral vascular involvement and more refractory disease compared to patients with LVV onset after 60.