Non-specific Aortoarteritis: difficulties in diagnosis, treatment, and outcome

Non-specific aortoarteritis in children is considered a rare disease, which is one of the reasons for its delayed diagnosis and treatment, leading to early disability and life-threatening complications, including death. Despite the active development of new diagnostic technologies and the widespread introduction of vascular ultrasound, there is still a trend toward late diagnosis of this disease. This is due to the non-specific nature of the symptoms, the ambiguous clinical picture and the delayed development of the characteristic symptoms of pulse absence and hypertension from the onset of the first symptoms.

The aim of the paper was to raise awareness among general practitioners and other non-rheumatologic specialists about the difficulties in detecting non-specific aortoarteritis in children and adolescents, its late diagnosis and consequent treatment failure, which significantly worsens the quality of life and prognosis of patients.

A retrospective analysis was performed using the medical records of children and adolescents (n = 17) under 17 years of age with non-specific aortoarteritis (Takayasu arteritis) who were evaluated and treated at the Scientific Center of Pediatrics and Pediatric Surgery in Almaty, Kazakhstan, from 2010 to February 2024. The study included 4 (23.5%) boys and 13 (76.5%) girls in a 1:4 ratio. The incidence of non-specific aortoarteritis in boys was mainly observed in early childhood, preschool and primary school age, whereas more than half of the girls showed the peak incidence in adolescence. Most of the affected children lived in rural areas, 12 (70.6%), and 5 (29.4%) children lived in urban areas. In terms of ethnicity, 15 (88.2%) patients were Kazakh, 1 (5.9%) patient was Uyghur (a 3-year-old female patient), and 1 (5.9%) patient was Russian (a 5-month-old patient).

The acute phase of non-specific aortoarteritis is characterized by nonspecific signs of systemic inflammation, leading to misdiagnosis such as acute respiratory viral infection or other infectious diseases, myocarditis, erythema nodosum, etc. When diagnosing non-specific aortoarteritis, general practitioners and non-rheumatologic specialists are guided by pulse absence in the radial arteries. However, in types III and IV of non-specific aortoarteritis, the subclavian arteries from the aortic arch are not affected. Pulses in the radial arteries are preserved. Non-specific aortoarteritis continues to be diagnosed late, leading to treatment failure, early childhood disability and poor prognosis.