Classification of Vasculitis in Childhood

2016-03-30T16-28-47Z
Source: Annals of Paediatric Rheumatology
Ezgi Deniz Batu, Yelda Bilginer.
Vasculitides are heterogeneous diseases characterized by inflammation in the vessel wall. The primary systemic vasculitides in childhood are quite uncommon except for Ig A vasculitis/Henoch-Schönlein purpura and Kawasaki disease. Besides the relative frequency of vasculitis subtypes, clinical characteristics and prognosis also tend to differ between children and adults. Because of the heterogeneous nature of vasculitis, it is difficult to identify proper subgroups. The nomenclature systems (names and definitions), classification, and diagnostic criteria are used to categorize vasculitides and these have evolved and have been revised with the substantial improvement in our understanding of vasculitis pathogenesis through advanced diagnostic tests and genetic studies. In the process of revision, the eponyms were replaced by more descriptive terms, new vasculitis categories were defined, the radiologic technologies and biomarkers (such as acute phase reactants and anti-neutrophil cytoplasmic antibodies) were incorporated, and the categorization depended on vessel size, type, and etiopathogenesis. The Chapel Hill Consensus Conferences 2012, the American College of Rheumatology criteria and the Ankara 2008 criteria constitute the major attempts for categorization and classification of vasculitides. The Diagnostic and Classification Criteria for Vasculitides study is currently underway to develop and validate diagnostic criteria and to improve classification criteria. Further multicenter prospective studies will help to incorporate the classification systems more into the clinical practice. Here, we review the current nomenclature, classification system, and classification criteria of vasculitides especially in childhood.


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