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

Highlights

• LVV_50-60 have more frequent aorta and peripheral limb involvement.

• LVV_50-60 have more refractory disease.

• LVV_50-60 should be probably treated more aggressively and monitored for aortic complications.

Abstract

Background

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.

Methods

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.

Results

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.

Introduction

The 2012 International Chapel Hill Consensus Conference (CHCC2012) subdivides vasculitides based on a combination of features, including the type of vessel affected [1]. Thereby, primary vasculitides may be distinguished into large vessel vasculitis (LVV), medium vessel vasculitis and small vessel vasculitis [1]. Primary LVV, involving the aorta and its major branches, are represented by two major variants, i.e. Takayasu arteritis (TA) and giant cell arteritis (GCA). According to the CHCC2012, histopathologic features of TA and GCA are indistinguishable, and TA is usually considered to predominantly involve young individuals whereas GCA predominantly involve older individuals [[2], [3], [4], [5], [6]].Thus, the age at onset of LVV is commonly used to distinguish both diseases, TA occurring before 50 years and GCA after 50 years [[7], [8], [9]]. However, GCA incidence peaks at 70–79 years [2,10,11], and only very few GCA appear before 60 years. In contrast, peak age for TA onset is usually between 20 and 30 years and the disease less commonly occurs after 50 years [6,12]. Therefore, patients diagnosed with LVV between 50 and 60 years of age can be difficult to classify.

Treatment of active forms of LVV is based on glucocorticoids, but therapeutic regimens that could be used in case of relapsing or refractory disease may differ between TA and GCA. Tumor necrosis factor (TNF)-α blockers were constantly found to be ineffective in prospective trials in GCA [[13], [14], [15]], whereas they showed some efficacy in retrospective studies in TA [[16], [17], [18], [19]]. In contrast, tocilizumab, an anti-interleukin (IL)-6 receptor monoclonal antibody, was shown to be effective in GCA in a large prospective randomized trial [20], whereas data from a small prospective trial failed to achieve the primary endpoint in TA but tended to favour tocilizumab [21,22].

In the present study, we describe the clinical pictures and outcomes of LVV occurring between the ages 50 and 60 (LVV_50–60) years, and compares them to LVV diagnosed after 60 years (LVV_>60).