Unclassified vasculitis

CER4516
2011 Vol.29, N°1 ,Suppl.64
PI 0081, PF 0085
Review

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PMID: 21470493 [PubMed]

Received: 08/02/2011
Accepted : 25/02/2011
In Press: 11/05/2011
Published: 11/05/2011

Abstract

Vasculitides are a heterogeneous group of inflammatory disorders of the blood vessels. The etiology and pathogenesis of vasculitides is incompletely understood, and the nomenclature and classification of vasculitides remains a challenge. A number of vasculitides were not included in the Chapel Hill Consensus (CHC) Conference definitions, thus, have remained `unclassified`, but may be included in a revised version of the nomenclature, e.g. Goodpasture`s syndrome. In other cases the term `unclassified` implies `unclassifiable`, i.e. a vasculitis cannot be assigned to any of the known entities. Vasculitis-induced acral necrosis including giant cell arteritis of small arteries as well as isolated forms of intestinal vasculitis including granulomatous giant cell polyphlebitis may belong to this category of rare `unclassified` vasculitides. In some entities the relationship between vasculitis and other manifestations remains unclear, e.g. in Behçet`s disease and IgG4-related systemic disease. In this review the clinical and pathological aspects of `unclassified` vasculitides are briefly discussed.