Abstract
BackgroundThis study aimed to identify new characteristics of elderly onset large-vessel vasculitis (EOLVV) by focusing on human leucocyte antigen (HLA) genotype, polymyalgia rheumatica (PMR), and affected vascular lesions observed on positron emission tomography/computed tomography (PET/CT) imaging.MethodsWe retrospectively studied 65 consecutive Japanese patients with large-vessel vasculitis (LVV) who had extracranial vasculitis lesions and underwent PET/CT imaging. PET/CT images were assessed using the semi-quantitative PET visual score of each affected vessel, and the PET vascular activity score (PETVAS) and number of affected vessels were calculated. Subjects were subsequently grouped based on age at onset, superficial temporal artery (STA) involvement, and presence of PMR and compared each group according to HLA genotype. Unsupervised hierarchical cluster analysis was used to identify the patients with similar characteristics in terms of affected vascular lesions detected through PET/CT imaging. The clinical characteristics and PET/CT findings of the population newly identified in this study were examined.ResultsTwenty-seven patients with EOLVV did not meet the American College of Rheumatology 1990 criteria for giant cell arteritis (GCA) and Takayasu arteritis and were considered as unclassified EOLVV (UEOLVV). The unsupervised hierarchical cluster analysis revealed that UEOLVV with PMR and large-vessel GCA (LV-GCA) formed a cluster of LVV with GCA features (i.e., PMR and/or STA involvement) when restricted to patients who were HLA-B52-positive. Patients who were HLA-B52-positive with LVV and GCA features had similar clinical characteristics and patterns of affected vessels and presented with diffuse LVV lesions. HLA-B52-positive patients who had LVV with GCA features also presented with higher PETVAS, more affected vessels, and lower rates of biologics usage and relapse compared to HLA-B52-positive patients with TAK.ConclusionsPatients who had UEOLVV with PMR had similar characteristics to patients with LV-GCA. Patients who were HLA-B52-positive and had LVV with GCA features presented with diffuse vascular lesions and may comprise a core population of Japanese patients with EOLVV. The findings of HLA-B52 positivity and diffusely affected vessels in patients with EOLVV can be considered as suspicious findings of LV-GCA.
Highlights
This study aimed to identify new characteristics of elderly onset large-vessel vasculitis (EOLVV) by focusing on human leucocyte antigen (HLA) genotype, polymyalgia rheumatica (PMR), and affected vascular lesions observed on positron emission tomography/computed tomography (PET/CT) imaging
Laboratory, and PET/CT findings between patients who had unclassified EOLVV (UEOLVV) with PMR and those with LV-giant cell arteritis (GCA) To classify EOLVV based on the HLA genotype, presence of PMR, and affected vascular lesions detected through PET/CT imaging, we initially focused on PMR, subsequently classifying the 27 patients with UEOLVV into two groups: 11 and 16 patients with and without PMR (Fig. 1)
Unsupervised hierarchical cluster analysis with Ward’s method was performed using the affected vascular lesions detected through PET/CT imaging in patients with newly diagnosed and untreated EOLVV, focusing on HLA-B52, which showed a difference in the frequency among patients with EOLVV
Summary
This study aimed to identify new characteristics of elderly onset large-vessel vasculitis (EOLVV) by focusing on human leucocyte antigen (HLA) genotype, polymyalgia rheumatica (PMR), and affected vascular lesions observed on positron emission tomography/computed tomography (PET/CT) imaging. Large-vessel vasculitis (LVV) can be generally categorized into giant cell arteritis (GCA) and Takayasu arteritis (TAK) based on the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides [1]. Both diseases usually differ according to age of onset and distribution of affected vessels. GCA has been classically known as a disease involving vasculitis of the cranial region, the temporal arteries (known as cranial GCA), with an age of onset over 50 years [1, 2]. In recent years, large-vessel GCA (LV-GCA), characterized by vascular lesions outside the cranial region, has been widely recognized as a subtype of GCA [2]. Distinguishing LV-GCA without superficial temporal artery (STA) involvement from TAK using conventional classification criteria has been exceedingly difficult [7, 8]
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