Vasculitis in children: Classification and incidence
- David Cabral, MBBS, FRCPC
David Cabral, MBBS, FRCPC
- Clinical Professor of Pediatrics, Head, Division of Rheumatology, Department of Pediatrics
- University of British Columbia
- Kimberly Morishita, MD, MHSc, FRCPC
Kimberly Morishita, MD, MHSc, FRCPC
- Clinical Assistant Professor of Pediatrics
- Division of Rheumatology, Department of Pediatrics
- University of British Columbia
Vasculitides are disorders defined by the presence of inflammation in a blood vessel wall (vasculitis). The inflammation may occur as a primary process or secondary to an underlying disease. Clinical symptoms vary widely depending upon the types and location of the vessels involved, the extent of inflammation, and subsequent vessel wall damage with associated hemodynamic changes.
The primary vasculitides may be classified according to their clinical manifestations, the size and type of the involved blood vessels, and the pathologic changes found within the vessel walls .
The nomenclature and criteria used for classification of the primary childhood vasculitides will be reviewed here. The approach to the child with suspected vasculitis and an overview of the management of children with vasculitic disorders are presented separately. The classification and approach to vasculitides in adults are also discussed elsewhere. (See "Vasculitis in children: Evaluation" and "Vasculitis in children: Management overview" and "Overview of and approach to the vasculitides in adults".)
Vasculitis is rare in children as demonstrated by the following two population-based studies:
●In an English survey of family clinicians, the estimated overall annual incidence of new cases of vasculitis was 53.3 per 100,000 children under 17 years of age . The two most common vasculitides were Henoch-Schönlein purpura (immunoglobulin A [IgA] vasculitis) and Kawasaki disease, with estimated annual incidences of 20.4 and 5.5 per 100,000 in children less than 17 years of age, respectively.
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- CLASSIFICATION VERSUS DIAGNOSIS
- CLASSIFICATION SYSTEMS
- CLASSIFICATION CRITERIA: MAJOR PRIMARY VASCULITIDES
- Henoch-Schönlein purpura (IgA vasculitis)
- Kawasaki disease
- Polyarteritis nodosa
- Infantile polyarteritis nodosa
- Cutaneous polyarteritis nodosa
- Takayasu arteritis
- Granulomatosis with polyangiitis (Wegener's)
- OTHER CHILDHOOD PRIMARY VASCULITIDES
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
- Hypersensitivity vasculitis
- Behçet's syndrome
- Primary angiitis of the central nervous system
- SECONDARY VASCULITIS
- MONOGENIC VASCULITIS