Takayasus Arteritis in Children: A Developing World Perspective

Received: Nov 25, 2013 Accepted: Dec 03, 2013 Ann Paediatr Rheum 2013;2:134-146 DOI: 10.5455/apr.120320130832 Introduction Takayasus Arteritis (TA) is a large vessel granulomatous vasculitis affecting the Aorta and its main branches. This disease has remained enigmatic since its ocular signs were first described in a young woman, by Japanese ophthalmologist Mikito Takayasu in 1908 [though it has been suggested that the first case was described earlier, in 1835 by another Japanese author, also in a young woman) [1]. Ironically the description by Takayasu himself did not mention involvement of the aorta or great vessels. The cause of TA is unknown but this disease demonstrates multifactorial aetiology, as it occurs with different clinical features, different infectious associations and with varying degrees rarity in different population groups. This review will consider general aspects of TA and highlight some aspects which are important from a developing world perspective. Epidemiology Takayasus Arteritis is a disease of younger people with an increased prevalence in females, though the true incidence and prevalence in children is not known. While it has been described in all populations, it does appear to occur with increased frequency in persons of Asian, South East Asian and African descent. Studies indicate incidences ranging from 2,6/million in the Olmsted County USA to 0,4 per million in Norwich, United Kingdom [2,3]. Given the nature of the disease, with protean and non-specific symptoms in the absence of end organ damage, these figures are almost certainly an underestimation. This is supported by one post mortem study from Japan, which showed sings of Takayasus in 1 per 3000 autopsies [4]. In our experience TA is also much more prevalent in South African children, yet no formal epidemiological studies have been published in this regard. Abstract