Page 160 - The Vasculitides Volumes 2

136 Patricia K. Coyle

includes IV ganciclovir, foscarnet, or a combination of both. One recently described elderly
woman with CMV encephalitis [54] later developed a post-infectious primary CNS vasculitis
[54].

Herpes Simplex and Epstein-Barr Virus
Herpes simplex virus (HSV), types 1 and 2, and Epstein Barr virus (EBV) have been

associated with CNS vasculitis [55, 56], however vessel wall contrast enhancement may be a
clue in suspected patients [56] and a positive findings in CSF PCR is adequate to justify
antiviral therapy. However, in contrast to VZV where reactivation is the mechanism of
causation, CNS vasculitis may be problematic due to latency of infection.

Retroviruses

Human Immunodeficiency Virus
Approximately 1% to 5% of individuals infected with human immunodeficiency virus

(HIV) are at risk of developing a stroke due to opportunistic infections, coagulopathy,
cardioembolism, HIV-associated vasculopathy and frank vasculitis [57]. Moreover, HIV-
associated arterial vasculitis is believed to account for 13% to 28% of ischemic strokes. CNS
vasculitis which is estimated to occur in less than 1% of cases of HIV infection is a diagnosis
of exclusion. Typically patients are in an advanced stage of the infection.

HIV can be associated with a granulomatous inflammation involving small arteries and
veins within the brain and leptomeninges. HIV patients with vasculitis should be assessed for
cryoglobulins and accompanying infection especially TB, syphilis, CMV, VZV, hepatitis B
and C virus (HBV and HCV), or a drug induced vasculitis.

Therapy typically involves highly active antiretroviral therapy (HAART), with
corticosteroids reserved for refractory cases due to vasculitis.

HTLV-1
Human T-cell lymphotropic virus-type 1 (HTLV-1) was the first described human

retrovirus. It causes adult T-cell leukemia and lymphoma, as well as a progressive
myelopathy referred to as tropical spastic paraparesis-HTLV-1 associated myelopathy in less
than 1% of infections. Neurological syndromes associated with HTLV-1 infection appear to
be due to active and selective expansion of retrovirus infected T-cells that harbor provirus that
selectively express HTLV-1 proteins such as Tax. In particular, activated cytotoxic CD8+ T
cells are increased. Perivascular inflammation is a frequent histopathological feature [58].
Although HTLV-1 is a recognized cause of retinal vasculitis [59, 60] there are rare associated
cases of CNS vasculitis [61]. The laboratory diagnosis of HTLV-1 infection is based on
serological and PCR studies. There is no proven antiviral therapy.

Hepatitis Agents

Hepatitis C is estimated to affect 170 million people worldwide with extrahepatic
involvement that occurs in 38% to 74% of cases [62]. Hepatitis C is associated with

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