Page 213 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Classification and Pathogenicity of ANCA-Associated Vasculitis 189

variable clinical presentation and absence of diagnostic criteria often leads to a delay in
diagnosis and initiation of effective therapy. The pathogenesis of AAV is complex involving
both the innate and adaptive immune systems.

Significant advances have been made in understanding the AAV over the past few
decades notably with development of animal models. Future advances will no doubt guide the
development of effective treatment approaches and preventative strategies.

References

[1] Watts, R. A., Gonzalez-Gay, M. A., Lane, S. E., et al. Geoepidemiology of systemic
vasculitis: comparison of the incidence in two regions of Europe. Ann. Rheum. Dis.
2001; 60:170-172.

[2] Watts, R., Lane, S., Bentham, G., et al. Epidemiology of systemic vasculitis. A
Ten-Year Study in the United Kingdom. Arthritis Rheum. 2000; 43:414-419.

[3] Booth, A. D., Pusey, C. D., Jayne, D. R. Renal vasculitis--an update, In: Nephrol. Dial.
Transplant. 2004; 19:1964-1968.

[4a] Jennette, J. C., Falk, R. J., Andrassy, K., et al. Nomenclature of systemic vasculitides.
Proposal of an international consensus conference. Arthritis Rheum. 1994; 37:187-192.

[4b] 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.

[5] Jayne, D. Update on the European Vasculitis Study Group trials. Curr. Opin.
Rheumatol. 2001; 13:48-55.

[6] Jayne, D. The diagnosis of vasculitis. Best Pract. Res. Clin. Rheumatol. 2009; 23:445-
453.

[7] Lane, S. E., Watts, R. A., Shepstone, L., et al. Primary systemic vasculitis: clinical
features and mortality. QJM 2005; 98:97-111.

[8] Pakrou, N., Selva, D., Leibovitch, I. Wegener's granulomatosis: ophthalmic
manifestations and management. Semin. Arthritis Rheum. 2006; 35:284-292.

[9] Seo, P., Stone, J. The antineutrophil cytoplasmic antibody-associated vasculitides. Am.
J. Med. 2004; 117:39-50.

[10] Robinson, M. R., Lee, S. S., Sneller, M. C., et al. Tarsal-conjunctival disease associated
with Wegener's granulomatosis. Ophthalmology 2003; 110:1770-1780.

[11] Jordan, D. R., Addison, D. J. Wegener's granulomatosis. Eyelid and conjunctival
manifestations as the presenting feature in two individuals. Ophthalmology 1994; 101:
602-607.

[12] Hoffman, G., Kerr, G., Leavitt, R., et al. Wegener granulomatosis: an analysis of 158
patients. Ann. Intern. Med. 1992; 116:488-498.

[13] Stone, J. H. and Group, WsGETR. Limited versus severe Wegener's granulomatosis:
baseline data on patients in the Wegener's granulomatosis etanercept trial. Arthritis
Rheum. 2003; 48:2299-2309.

[14] Kornblut, A. D., Wolff, S. M., Fauci, A. S. Ear disease in patients with Wegener's
granulomatosis. Laryngoscope 1982; 92:713-717.

[15] Kempf, H. G. Ear involvement in Wegener's granulomatosis. Clin. Otolaryngol. Allied
Sci. 1989; 14:451-456.

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