Page 210 - The Vasculitides Volumes 2

186 Kelly G. Gwathmey, Jennifer A. Tracy and P. James B. Dyck

Conclusion

Peripheral nerve vasculitis is commonly encountered in patients with primary systemic
vasculitis, but may also be secondary to viral infections, malignancy, or connective tissue
disease. Primary and secondary systemic vasculitic neuropathies are forms of nerve large
arteriole vasculitis.

Non-systemic vasculitic neuropathy accounts for up to 30% of all vasculitic neuropathies.
Non-systemic vasculitic neuropathy, including some forms of secondary systemic vasculitic,
and the radiculoplexus neuropathies notably DLRPN, LRPN, DCRPN, and PDMN, the latter
of which are considered localized forms of vasculitic neuropathy, all associated with
microvasculitis. Treatment employing corticosteroids and other systemic immunosuppressive
agents, plasma exchange and intravenous immune globulin, are suitable options in most
patients with severe or progressive neurological deterioration to prevent excess morbidity and
mortality.

References

[1] Younger, D. S. Vasculitis of the nervous system. Curr. Opin. Neurol. 2004; 17:317-
336.

[2] Said, G., Lacroix, C. Primary and secondary vasculitic neuropathy. J. Neurol. 2005;
252:633-641.

[3] Schaublin, G., Michet, C. J., Dyck, P. J. B., et al. An update on the classification and
treatment of vasculitic neuropathy. Lancet Neurol. 2005; 4:853-865.

[4] Burns, T. M., Schaublin, G. A., Dyck, P. J. B. Vasculitic neuropathies. Neurol. Clin.
2007; 25:89-113.

[5] Lie, J. T. Nomenclature and classification of vasculitis: plus ça change, plus c?est la
meme chose. Arthritis Rheum. 1994; 37:181-186.

[6] Zeek, P. M. Periarteritis nodosa; a critical review. Am. J. Clin. Pathol. 1952; 22:777-
790.

[7] Dyck, P. J., Daube, J., O?Brien, P., et al. Plasma exchange in chronic inflammatory
demyelinating polyradiculoneuropathy. N. Engl. J. Med. 1986; 314:461-465.

[8] Dyck, P. J. B., Englestad, J., Dyck, P. Microvasculitis. In: Dyck, P., Thomas, P., eds.
Peripheral Neuropathy. 4th ed. Philadelphia: Elsevier; 2005.

[9] Collins, M. P., Dyck, P. J. B., Gronseth, G. S., et al. Peripheral Nerve Society Guideline
on the classification, diagnosis, investigation, and immunosuppressive therapy of non-
systemic vasculitic neuropathy: executive summary. J. Peripher. Nerv. Syst. 2010; 15:
176-184.

[10] Jennette, J. C., Falk, R. J., Bacon, P. A., et al. 2012 Revised International Chapel Hill
Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65:1-11.

[11] Wolf, J., Schmitt, V., Palm, F., et al. Peripheral neuropathy as initial manifestation of
primary systemic vasculitides. J. Neurol. 2013; 260:1061-1070.

[12] Lacomis, D., Zivković, S. A. Approach to vasculitic neuropathies. J. Clin.
Neuromuscul. Dis. 2007; 9:265-276.

Complimentary Contributor Copy