Page 163 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Nomenclature and Pathologic Features of Vasculitides 139

Figure 11. Primary central nervous system vasculitis (CNS single organ vasculitis) affecting a small
intracerebral artery with granulomatous inflammation resulting in perivascular hemorrhage (H & E
stain).

An extended period of surveillance is recommended to substantiate the accuracy of the
diagnosis of SOV and that excision of the affected site provides a cure [37]. Moreover,
CHCC 2012 recommends that the term SOV not be used for organ-limited expressions of
vasculitides that usually have multisystem involvement, for example GPA confined to the
lungs or lung-limited GPA; or ANCA-positive pauci-immune necrotizing glomerulonephritis
without systemic vasculitis also known as renal-limited-AAV.

Vasculitis Associated with Systemic Disease

CHCC 2012 acknowledges that vasculitis can be associated with or secondary and caused
by different systemic diseases [1]. Therefore the name or diagnosis should include a prefix
term specifying the systemic disease such as RV, lupus and sarcoid vasculitis (Figure 12).
There is a subjective distinction between vasculitis secondary to a systemic inflammatory
disease and vasculitis that is characteristic of a systemic inflammatory disease. In some
instances, it may be a relatively infrequent feature of a systemic disease as for example
vasculitis in rheumatoid arthritis, or a frequent feature and therefore characteristic component
of the systemic disease, such as in GPA; however in CS, vasculitis falls into a gray zone.
The important concept is that whenever a vasculitis is identified, the possibility of vasculitis
secondary to a systemic disease should be considered.

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