Pediatric Vasculitides: A Generalists Approach

Abstract

Vasculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. Primary vasculitides are rare in children. These are defined by both the size of vessels involved and the type of inflammatory response. Clinical features consist of multi-organ involvement on a background of constitutional features reflecting the size and location of the blood vessels involved. Whilst some vasculitides are best diagnosed clinically, many forms require sophisticated imaging and other investigations (auto antibodies) to reveal the correct diagnosis. Prompt recognition and treatment is crucial as many of the vasculitides cause significant morbidity or mortality. Treatment options range from symptomatic therapy, immunosuppresive agents, intravenous immunoglobulin (IVIG) or biologic agents and are determined by the type of vasculitis, the severity of the inflammation, and the organ systems affected. Early detection and aggressive treatment is crucial for the best outcomes in the most severe forms of childhood vasculitis.

Exantemas escarlatiniformes en la infancia

“…Sin embargo, se considera de buen pronóstico en general y solo 1 a 2 % muere agudamente por infarto de miocardio. Además, se debe tener en cuenta que puede alcanzar tasas de recurrencia hasta de 3 a 4% 4,28,31,32 La enfermedad de Kawasaki tiene distribución mundial, afectando, al menos, 10 a 20 niños de cada 100.000 por año, aunque su prevalencia depende de la raza 32 . La edad de presentación más frecuente es entre los seis meses y los cinco años, con pico entre los 9 y 11 meses.…”

“…Cuando se detecte alguna anomalía, se debe repetir al año. Por otro lado, la angiografía brinda poca información sobre el flujo coronario, mientras que la tomografía axial en espiral y la resonancia magnética tienen una resolución limitada 32 .…”

Pattern of Paediatric Rheumatic Diseases: An Experience in a Tertiary Care Hospital, Dhaka, Bangladesh

“…5 Vasculitis in children appears to have an incidence of about 50 cases per 100,000 children per year. 6 In Bangladesh no study on the incidence and prevalence of PRDs had been done. A small community based study found that prevalence of JIA was 60 per 100000 children in rural Bangladesh 7 .…”

Primäre Vaskulitiden im Kindesalter

“…sollte an eine Vaskulitis gedacht werden[2,3].Typisch für HSP und KS (akute Vaskulitiden) im Kindesalter ist die Hautmanifestation (Purpura, generalisiertes Exanthem). Dementsprechend sollte die Diagnose relativ rasch klinisch gestellt werden können.…”