The Severity of Takayasu Arteritis Is Associated with the HLA-B52 Allele in Japanese Patients

Abstract

Takayasu arteritis (TA) is a type of vasculitis that affects the large elastic arteries, specifically the aorta and its main branches. It has been reported that TA occurred most frequently in Nagasaki Prefecture, the western area in Japan. We retrospectively collected the information of 34 patients with TA, diagnosed using the American College of Rheumatology 1990 criteria for the classification of TA, from the medical records of Nagasaki University Hospital from 2003 to 2015, and we investigated the clinical characteristics of these TA patients. Among the 35 patients, 25 patients were examined for the existence of the HLA-B52 allele that has been reported to influence TA susceptibility. Seventeen patients (68.0%) of the 25 patients were HLA-B52-allele-positive, which was defined as the state of having at least one HLA-B52 allele. There was a significant difference in the rate of smokers: HLA-B52-allele-positive: six patients (35.3%) vs. HLA-B52-allele-negative: 0 (0.0%). The C-reactive protein level in the HLA-B52-positive patients (9.0 ± 6.4 mg/dL) was significantly higher than that in the HLA-B52-negative patients (3.2 ± 3.9 mg/dL). All HLA-B52-allele-positive patients were found to be active according to Kerr's criteria. The HLA-B52-positive patients' initial prednisolone dosage (37.7 ± 8.6 mg/day) was significantly higher than that of the HLA-B52-allelenegative patients (23.1 ± 13.1 mg/day). Thus, the HLA-B52 allele is associated with the disease activity and the steroid requirements of TA patients. Furthermore, our present findings have revealed for the first time that the HLA-B52 allele and smoking might be associated with the onset of TA.

Presentation and Disease Course of Childhood‐Onset Versus Adult‐Onset Takayasu Arteritis

“…Various studies have shown the critical role of specific cytokine profiles and immunologic pathways in the promotion of vascular inflammation and disease activity in patients with TAK . Furthermore, genetic factors are known to play an important role in the pathophysiology of TAK . However, none of these potential causes underlying the differences in disease activity between children and adults with TAK have yet been evaluated in comparative studies.…”

A familial case of takayasu arteritis: HLA-related genetic susceptibility and efficacy of anti-TNFα treatment in two italian siblings

“…On the basis of these findings, the diagnosis of TA was confirmed in june 2014. The allele HLA-B52 is the most frequently and strongly associated with TA in Japan and other Asian countries [8,9] and relates with worse clinical manifestations, higher rate of vascular complications and poor prognosis [10,11]. According to literature findings, our patients presented severe features suggesting a bad prognosis: the disease onset was underhand but the progression of thickening of the arterial walls was rapid and diffuse, leading to severe carotid occlusion and involving also the aortic bulb in case 2.…”

“…The main reported association is with HLA-B5 and especially with its split B52 [8][9][10][11]. The allele B51 is also considered a possible gene of susceptibility but the evidence is weaker [12].…”

New Insights on the Pathogenesis of Takayasu Arteritis: Revisiting the Microbial Theory

“…Despite TAK primarily affecting young or middle-aged women, particularly from Asian countries, the disease has a worldwide distribution and has been reported in people of virtually all ages [ 11 ]. So far, the only genetic factor that shows a consistent association with TAK is the HLA allele, HLA-B*52, which has been confirmed in various cohorts and in several ethnicities [ 12 , 13 , 14 ]. Thus, it has been proposed that the increased prevalence of TAK in Asians may reflect the higher frequency of the HLA-B*52 in this population.…”