Page 145 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

In: The Vasculitides, Volume 1 ISBN: 978-1-63463-110-5
Editors: David S. Younger © 2015 Nova Science Publishers, Inc.

Chapter 6

Nomenclature and Pathologic
Features of Vasculitides

J. Charles Jennette?, M.D., Ronald J. Falk, M.D. and
Adil. H. M. Gasim, M.D.

Department of Pathology and Laboratory Medicine, University
Of North Carolina at Chapel Hill, Chapel Hill, NC, US

Abstract

The goal of this chapter is to establish a general framework for categorizing
vasculitis, which also serves as a framework for diagnosing vasculitis. The basis for the
names and definitions used in this chapter is the 2012 Chapel Hill consensus conference
nomenclature system for vasculitides. The major subdivisions are large vessel vasculitis,
medium vessel vasculitis, small vessel vasculitis, variable vessel vasculitis, single-organ
vasculitis, vasculitis associated with systemic disease, and vasculitis associated with a
probable etiology. Although the names of the first four categories imply that vessel
diameter is a defining feature of many forms of vasculitis, the different functional and
structural characteristic of vessels are more important than the diameter of the vessel in
determining their involvement in different forms of vasculitis, as well as the nature of the
pathogenic events that are inducing the inflammation. Vasculitides that will be defined in
this chapter include Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Kawasaki
disease, microscopic polyangiitis, granulomatosis with polyangiitis (Wegener),
eosinophilic granulomatosis with polyangiitis (Churg-Strauss), anti-glomerular basement
membrane disease, cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schönlein),
hypocomplementemic urticarial vasculitis (anti-C1q vasculitis), Behçet disease and
Cogan syndrome. Many factors must be taken into consideration to make an accurate and
precise clinical diagnosis of vasculitis. These include the type of vessels affected, as for
example arteries versus venules; the histopathologic features of the inflammatory process
such as leukocytoclastic inflammation or granulomatous inflammation; the
immunopathologic features that may include IgA-dominant deposits or a paucity of
immunoglobulin in vessel walls; the serologic finding of cryoglobulins or anti- neutrophil

? Corresponding author: J. Charles Jennette, M.D. Brinkhous Distinguished Professor and Chair, Room 308
Brinkhous-Bullitt Building, Department of Pathology and Laboratory Medicine, University of North Carolina
at Chapel Hill, Chapel Hill, NC 27599. E-mail: [email protected].

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