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AB0566 Outcomes of Takayasu's Arteritis in Children and Adolescents
  1. R. Maytbasova,
  2. P. Ishuova
  1. Scientific Center of Pediatrics and Children's Surgery, Almaty, Kazakhstan

Abstract

Background Takayasu's arteritis (AT) in children and adolescents is ranked number third in frequency among the vasculitis and is one of the common causes of renovascular hypertension. It is believed that the sickness rate of AT is more common in Asian ethnic group. In this disease, vascular stenosis has been found in 90% of cases and 45% of them is the aneurysm. In this regard, early diagnosis and adequate treatment are important to avoid the development of stenosis, aneurysms, vascular thrombosis, ischemia of organs and an adverse outcome.

Objectives To conduct a retrospective analysis of the course and survival in children and adolescents with Takayasu's arteritis.

Methods A retrospective analysis of the course and survival in patients with Takayasu's arteritis observed in the clinic of the Institute since 2005 was conducted. Total: 9 patients. Age: 10–17 years. The ratio of girls/boys was 3.5:1. Takayasu's arteritis was diagnosed according to the diagnostic criteria recommended by the EULAR/ PRINTO/ PRES (2010).

Results All patients were patients of Kazakh nationality. None of relatives of the 1st and 2nd relationship degree had any cases of vasculitis or autoimmune diseases. Chronic nasopharyngeal infection, carious teeth were identified in all patients, which could be a trigger factor for the occurrence of Takayasu arteritis. But, no any cases of relation between Takayasu arteritis and TB infection were identified. On average, from the appearance of disease symptoms and before diagnosis of the disease 14 months had left (8 months ÷ 2 years 2 months). In all patients, Takayasu's disease was diagnosed at advanced stage, characterized by lesions of the arteries extending from the aorta, which was confirmed by angiography. Vascular disease Type III was revealed in 7 patients, type IV in 2. Secondary shrinkage of both kidneys due to a complete occlusion of the renal arteries developed in one 13 years old patient. All patients had undertaken active anti-inflammatory (glucocorticoids) and immune suppressive therapy (methotrexate 10–15mg/m2, tocilizumab 12 mg/kg), symptomatic therapy.

During the therapy, unstable and partial regression of general inflammatory symptoms were observed, hypertension was not completely stopped in 8 patients (88.9%), including a patient with end-stage of renal failure. Given the nature (III and type IV) of vascular lesions, unstable and partial anti-inflammatory effect of the therapy, the prognosis for life was unfavorable. Since diagnosis, survival rate in patients under 7 years was 22.2% (2), under 5 years – 44.4% (4) under 2 years – 22.2% (2) and at one year – 11.1% (1). The cause of death was: end stage of renal disease – 11.1% (1), stroke – 55.5% (5), myocardial infarction – 22.2% (2) and aortic aneurysm rupture of 11.1% (1).

Conclusions Thus, the diagnosis of AT in children and adolescents was revealed late, which delayed an early prescription of adequate immunosuppression and contributed to the development of severe complications and death

Disclosure of Interest None declared

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