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Diseases of the Aorta
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Takayasu arteritis (TA) is an idiopathic giant cell granulomatous vasculitis of the aorta and its main branches which constitutes one of the more common vasculitides in younger patients under the age of 50 and is more frequent in females. TA is rare but it is the commonest large vessel vasculitis in children, representing the leading cause of stenotic aorto-arteriopathy and one of the most prevalent causes of reno-vascular hypertension in childhood. Childhood TA is associated with mortality rates as high as 35%. TA has been recognized worldwide. Its overall incidence has been estimated to be 2/1 000 000 per year. It is more prevalent in Central and South America, Africa, India and the Far East. The aetiology of TA remains poorly understood, but genetic contribution to the disease pathogenesis is supported, mainly by its association with the HLA complex. HLA associations are varied and differ according to the patients’ ethnic background. The strongest association has been established with HLA-B52 in Japanese and other populations.
Updates in the diagnosis and management of Takayasu’s arteritis
Published in Postgraduate Medicine, 2023
Anupam Somashekar, Yiu Tak Leung
The etiology of Takayasu’s arteritis is unknown; however, multiple contributing genetic and environmental factors have been explored. The association between human leukocyte antigen (HLA) B-52 and Takayasu’s arteritis has been well described through the literature, first genotyped by Isohisa et al. in 1978 [14]. Further studies in Japan have also demonstrated an association with multiple HLA alleles including HLA A9 [14] and HLA B67 [15]. However, HLA B52 is the only genotype to extend to multiple ethnicities, including European American, Turkish, and Asian populations [16]. Patients who test positive for HLA B52 have been found to have increased incidence of aortic regurgitation [17–19], steroid refractory disease, and disease recurrence [18]. In addition to HLA B52, non-HLA sites have been reported as susceptibility loci, with the most reported being the IL-12B locus, which encodes for interleukin 12 [20]. A recent genome wide association study identified over 60 loci which were associated with Takayasu’s arteritis and created a genetic risk score, which may help counsel patients in the future regarding their risk to develop the disease [21]. Of note, this study identified that Takayasu’s arteritis demonstrated the closest genetic relatedness to inflammatory bowel disease.
The feasible maintenance dose of corticosteroid in Takayasu arteritis in the era of biologic therapy
Published in Scandinavian Journal of Rheumatology, 2021
T Shirai, H Sato, H Fujii, T Ishii, H Harigae
The differences in the response to treatment suggest the heterogeneous nature of TAK, although the presentation is commonly aortitis. The causes of aortitis include primary LVV infection and drugs, including TNF inhibitors and granulocyte colony-stimulating factor (23). In particular, the association of TAK with human leucocyte antigen (HLA)-B52, HLA-B67, and interleukin-12B has been reported (24). As effector cells, immune cells, including macrophages, T cells, and B cells, also play critical roles (25, 26). In 2020, we identified novel autoantibodies, anti-EPCR antibody, and anti-SRBI antibody in TAK; thus, TAK seems to be constituted by several subgroups (27). Therefore, it is important to investigate the response to treatments from the viewpoint of these different subgroups of TAK in the future.
Large-vessel giant cell arteritis eleven months after a diagnosis of erythema nodosum
Published in Modern Rheumatology Case Reports, 2020
Takahiro Tsuji, Kotaro Kunitomo
It is important to differentiate GCA from TAK because they share many common features. Their common clinical, histopathological and radiographic features suggest that these two conditions are a spectrum of the same disease, the phenotype of which may be influenced by age [8]. Although temporal artery biopsy is important for the diagnosis of GCA, its sensitivity is limited [9]. HLA-A24, B52 and DR2 are generally associated with Japanese TAK patients and HLA-B52- or B67- positive tests are useful to make a differential diagnosis of TAK and GCA as described in the 2017 Japanese guideline for TAK. However, negative findings are not useful for a differential diagnosis. We differentiated GCA from TAK based on the case’s age at onset. On the other hand, this case was not a typical cranial GCA because it was not confirmed by biopsy whether or not the temporal artery was affected, and there were no symptoms of polymyalgia rheumatica (PMR) or jaw claudication; therefore, it could be diagnosed as elderly-onset TAK. However, the similarities and differences between LV-GCA and elderly-onset TAK are controversial.