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Peripheral Nerve Vasculitis: Classification and Disease Associations 185

It is also effective in patients with CV and RV [66, 127-133]. Nearly 90% of patients with
treatment-refractory AAV improved following rituximab treatment in uncontrolled series
[67]. Plasma exchange is an option in patients with fulminant vasculitis and significant organ
impairment, as well as, in HBV-associated PAN, HCV-associated CV, and HIV-associated
systemic vasculitis [134-138].

Treatment of HCV-associated CV consists of antiviral therapy with pegylated interferon-
? and ribavirin, but rituximab may be more effective in patients with severe cryoglobulinemic
vasculitis [66, 67, 129, 139]. For non-cytomegalovirus-related HIV vasculitis, treatment is
similar to HBV-associated PAN using antiviral therapy and plasma exchange, although in
clinical practice most use corticosteroids [140, 141]. The primary treatment for HBV-related
PAN is antiviral therapy as corticosteroids may activate viral replication and accelerate
hepatic damage [114, 142, 143].

The approved treatments for chronic HBV include interferon-?, pegylated interferon-?,
and oral nucleoside/nucleotide analogs. The French Vasculitis Study Group [60, 138]
treatment protocol for HBV-related PAN that included antiviral agents, therapeutic plasma
exchange to remove circulating immune complexes followed by two weeks of corticosteroids
to treat the severe symptoms, induced complete remission in 80% to 90% of patients.

The 2010 Peripheral Nerve Society published guidelines for the immunosuppressive
therapy of classic NSVN [9] and deemed that patients with progressive symptoms and
biopsy-proven active vasculitis warranted treatment with immunosuppression while those
without biopsy-proven vasculitis, and others clinically stable and improving, should be
observed. While corticosteroid therapy is the first-line treatment in NSVN, the use of
combination therapy in patients with rapidly progressive NSVN is appropriate and supported
by two class III retrospective cohort surveys [22, 71].

Patients with progressive symptoms early in the course of DLRPN or LRPN are
candidates for immunotherapy [144]. One prospective, randomized, double-blind,
multicenter-controlled trial employing intravenous methylprednisolone in patients with
DLRPN found improvements in indices of pain but not neurological function [145].
However, that study may have been conducted too late in the disease course to affect
impairment. One open uncontrolled study employing intravenous methylprednisolone in
patients with clinically deteriorating LRPN, showed improvement in NIS commensurate with
clinical improvement in all patients [146, 147]. While case series of patients [147-149] with
radiculoplexus neuropathies suggest improvement in pain and strength with IVIg, there have
been no randomized trials of plasma exchange in DLRPN or LRPN. One case series of five
patients with DLRPN treated with plasma exchange demonstrated improvement [150].
Considering that DLRPN and LRPN are subtypes of NSVN, a similar treatment regimen for
patients with LRPN should be undertaken.

Pain control is of utmost importance in vasculitic neuropathy and the usual medications
employed include tricyclic antidepressants, pregabalin, gabapentin, serotonin-norepinephrine
reuptake inhibitors, valproate, carbamazepine, tramadol, topical lidocaine, topical capsaicin,
and narcotics [151]. Continued pain without progressive neurologic deficits is not an
indication for high-dose corticosteroids.

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