Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Primary large-vessel vasculitis (LVV) include giant cell arteritis (GCA) and Takayasu arteritis (TA). Age at onset is commonly used to distinguish GCA and TA. TA usually occurs before 50 years, whereas GCA occurs after age 50. However, GCA onset before age 60 remains very rare, reason why LVV between age 50 and 60 could be difficult to classify.
Methods: We conducted a national, retrospective study including patients with LVV, defined by histological (temporal artery biopsy) and/or imaging (circumferential thickening and/or hypermetabolism on FDG PET/CT) evidence of inflammatory vascular disease, and aged between 50 and 60 years at onset (LVV50-60). Cases were compared to controls with GCA aged over 60 years (LVV>60), and matched on gender with a ratio of 1:1.
We included 183 patients (136 women). Initial symptoms of LVV were: constitutional symptoms in 144 (79%) cases, cephalic symptoms in 133 (73%), polymyalgia rheumatica in 55 cases (30%), peripheral limb ischemic manifestations in 42 (23%), ocular signs in 32 (17%), stroke in 4 cases (2%) and mesenteric ischemia in 2 cases (1%). Temporal artery biopsy showed evidence of vasculitis in 78 (43%) cases.
Computed tomography (CT) angiography was performed in 102 (56%) cases and was abnormal in 74%, involving aorta in 83% (thoracic 29%, abdominal 8% and both 63%), subclavian artery in 26%, iliofemoral artery in 18% and carotid artery in 14%. Isolated aortitis was observed in 38%.
FDG PET/CT scan was performed in 105 (57%) cases, showing hypermetabolism in 90%. Overall, aortitis was noted on CT angiography and/or FDG-PET/CT in 113 (78%) cases, without any cephalic symptoms in 22%.
All patients received glucocorticoids. After a median follow-up of 43.8 months, 78 (31%) patients required second-line therapy, 27 (18%) three-line, and 14 (9%) more lines. Overall, 35% received methotrexate and 12% biological agent (anti-TNFa and/or IL-6 blockade). Fifteen patients required surgery (bypass surgery or angioplasty). At the end of follow-up, only 45% had discontinued glucocorticoids.
Case-control comparison showed that LVV50-60 had more frequent peripheral limb ischemic manifestations (23 vs 5%, P<0.0001), and less frequent cephalic symptoms (72 vs 90%, P<0.0001) and ocular signs (17 vs 27%, P=0.04). CT angiography and FDG PET/CT scan were more frequently abnormal in LVV50-60 (41 vs 23%, P<0.0001; and 51 vs 27%, P=0.007, respectively), with aorta being more frequently involved (78 vs 47%, P<0.0001). LVV50-60 received a median of 2 lines of treatment compared to one in LVV>60 (P=0.0002). LVV50-60 had more frequent surgery (10 vs 0%, P<0.0001), received more frequent biological agents (12 vs 3%, P=0.003), and had at last follow-up higher median prednisone dose (8.8 vs 6.5 mg/d, P=0.048) and lower frequency of patients with prednisone <7.5 mg/d (71 vs 83%, P=0.01) compared to LVV>60.
Conclusion: Primary LVV onset between 50 and 60 years identifies a subset of patients with more frequent aorta and peripheral limb vascular involvement compared to patients with LVV onset after 60. LVV between 50 and 60 were also characterized by more refractory disease requiring more methotrexate and/or biological agent.
To cite this abstract in AMA style:Delaval L, Daumas A, Samson M, Ebbo M, de Boysson H, Liozon E, Dupuy H, Regent A, Puyade M, Blockmans D, Lazaro E, Benhamou Y, Sacre K, Bérezné A, Guillevin L, Terrier B. Presentation and Outcome of Large-Vessel Vasculitis Diagnosed between 50 and 60 Years: Case-Control Study Based on 183 Cases [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/presentation-and-outcome-of-large-vessel-vasculitis-diagnosed-between-50-and-60-years-case-control-study-based-on-183-cases/. Accessed November 19, 2019.
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