Takayasu arteritis in children and adolescents

Abstract

Takayasu arteritis is a devastating vasculitis of the aorta and its major branches. The clinical manifestations in paediatric patients are less specific than in adults: in children the disease presents with fever, arthralgias and hypertension. Intramural inflammation results in narrowing of the blood vessel lumen and therefore hypoperfusion of the parenchyma. Conventional angiography is the gold standard diagnostic procedure. Corticosteroids, cyclophosphamide, MTX and biological therapies such as TNF-α blocking agents are treatment options.

Childhood‐onset Takayasu arteritis: an update

“…7,11 Hypertension (82.6%), headaches (31%), fever (29%), breathlessness (23%), weight loss (22%) and vomiting (20.1%) are described more commonly in the West. 2 Musculoskeletal symptoms vary from 14% to 65% in various series. 4 A series from the authors' centre depicts hypertension (73%), headache (53%), constitutional symptoms (53%) and fever (45%) as the more frequent manifestations.…”

“…However, the childhood onset TA (c-TA) subset affects any age group, from young infants to late adolescents, with the youngest reported patient being diagnosed at 6 months after birth. 2 It is reported as the third most common cause of vasculitis in the pediatric age group. Due to non-specific symptoms during the acute phase of disease and scarcity of validated biomarkers to assess its activity and damage, diagnosis of c-TA remains a daunting challenge to clinicians, explaining the worldwide paucity of data.…”

Pregnancy in Takayasu arteritis - maternal and fetal outcome

“…Cortico steroids are the main stay of therapy with remission rate up to 60%. [29][30][31] According to EULAR. European league against rheumatism, starting dose of glucocorticoids = 1mg/kg/bodyweight for 4 weeks and then taper off.…”

Pre-pulseless Takayasu Arteritis Presenting with Hypertension

“…The diagnosis of TA is therefore based on clinical and angiographic criteria. 17 Doppler ultrasound may be a useful non-invasive procedure for assessment of vessel wall inflammation, but angiography remains the goldstandard.…”

“…Due to the non-specificity of the symptoms and the absence of specific laboratory parameters, the disease is often unrecognized in this phase. 17 TA has been diagnosed at the pre-pulseless period after stenotic lesions of the aorta and its branches were detected on imaging, in case reports of two young females presenting with fever of unknown origin. 18 An absence of the physical findings related to vasculitic lesions, common in the pre-pulseless period, should not rule out the disease.…”