Vasculitis is a clinicopathologic process characterized by inflammation of and damage to blood vessels. The vessel lumen is usually compromised, and this is associated with ischemia of the tissues supplied by the involved vessel. A broad and heterogeneous group of syndromes may result from this process, since any type, size, and location of blood vessel may be involved. Vasculitis and its consequences may be the primary or sole manifestation of a disease; alternatively, vasculitis may be a secondary component of another disease. Vasculitis may be confined to a single organ, such as the skin, or it may simultaneously involve several organ systems.
A major feature of the vasculitic syndromes as a group is the fact that there is a great deal of heterogeneity at the same time as there is considerable overlap among them. This heterogeneity and overlap in addition to a lack of understanding of the pathogenesis of these syndromes have been major impediments to the development of a coherent classification system for these diseases. Table 385-1 lists the major vasculitis syndromes. The distinguishing and overlapping features of these syndromes are discussed below.
PATHOPHYSIOLOGY AND PATHOGENESIS
Generally, most of the vasculitic syndromes are assumed to be mediated at least in part by immunopathogenic mechanisms that occur in response to certain antigenic stimuli. However, evidence supporting this hypothesis is for the most part indirect and may reflect epiphenomena as opposed to true causality. Furthermore, it is unknown why some individuals might develop vasculitis in response to certain antigenic stimuli, whereas others do not. It is likely that a number of factors are involved in the ultimate expression of a vasculitic syndrome. These include the genetic predisposition, environmental exposures, and the regulatory mechanisms associated with immune response to certain antigens. Although immune complex formation, antineutrophil cytoplasmic antibodies (ANCA), and pathogenic T lymphocyte responses (Table 385-2) have been among the prominent hypothesized mechanisms, it is likely that the pathogenesis of individual forms of vasculitis is complex and varied.
TABLE 385-1Vasculitis Syndromes ||Download (.pdf) TABLE 385-1 Vasculitis Syndromes
|Primary Vasculitis Syndromes ||Secondary Vasculitis Syndromes |
Granulomatosis with polyangiitis (Wegener’s)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
IgA vasculitis (Henoch-Schönlein)
Giant cell arteritis
Cutaneous leukocytoclastic angiitis
Primary central nervous system vasculitis
Vasculitis associated with probable etiology
Hepatitis C virus–associatedcryoglobulinemic vasculitis
Hepatitis B virus–associatedvasculitis
Vasculitis associated with systemic disease
PATHOGENIC IMMUNE-COMPLEX FORMATION
Deposition of immune complexes was the first and most widely accepted pathogenic mechanism of vasculitis. However, the causal role of immune complexes has not been clearly established in ...