Page 65 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Overview of Primary and Secondary Vasculitides 41

specimens. The remaining two patients without definable vasculitis exhibited nonspecific
perivascular lymphocytic inflammation. A similar pathology was noted in the distribution of
large named cerebral vessels and single stenosis on cerebral angiography in a child with focal
seizures and large arterial ischemic lesions [192].

The approach to the patient with presumed PCNA has been reviewed [193]. It begins
with an accurate diagnosis and the exclusion of disorders that can mimic primary CNS
vasculitis including reversible cerebral vasoconstrictive vasculitis (RCVS) [194, 195]. The
evaluation is incomplete without sampling of the CSF which often demonstrates elevation of
the protein content and lymphocytic pleocytosis [193]. Cerebral angiography, high-resolution
MRA and CTA, all provide complementary information however a small vessel vasculitis
may escape detection, making it necessary to contemplate combined brain and meningeal
biopsy for tissue confirmation (Figure 4).

A

B

Reproduced from reference [4], with permission.
Figure 4. Central nervous system vasculitis. A. The media and adventitia of this small leptomeningeal
artery have been almost completely replaced by multinucleated giant cells (arrowheads). There is
intimal proliferation with obliteration of the vascular lumen, and a dense, perivascular, mononuclear
inflammatory infiltrate can be seen (stain, hematoxylin and eosin; original magnification, ×250). B. A
somewhat larger leptomeningeal vessel shows necrosis of the media and internal elastic lamina with
multinucleated giant cell formation (arrows), intimal proliferation (arrowhead), and lymphocytic
infiltration of the adventitia and neighboring meninges (stain, hematoxylin and eosin; original
magnification, ×250).

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