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CHaPtEr 58 Small- and Medium-Vessel Primary Vasculitis 805

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• Improved understanding of the underlying pathogenic mechanisms and giant cell arteritis between 1994 and 2006 in northern Germany.
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and increase the risk of hepatoma or induce the development of the 14. Primo VC, et al. Anti-PR3 immune responses induce segmental and
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• The discovery of the involvement of the complement pathway (C5) 18. Stassen PM, et al. HLA-DR4, DR13(6) and the ancestral haplotype
in AAV has led to the testing of therapies directed against C5a in A1B8DR3 are associated with ANCA-associated vasculitis and Wegener’s
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• The ultimate goal remains to identify upstream pathologies leading 19. Cao Y, et al. DRB1*15 allele is a risk factor for PR3-ANCA disease in
to vasculitis, which promises to fundamentally change the management African Americans. J Am Soc Nephrol 2011;22(6):1161–7.
of these devastating diseases, as it may permit the induction and 20. Papiha SS, et al. Association of Wegener’s granulomatosis with HLA
maintenance of drug-free remission.
antigens and other genetic markers. Ann Rheum Dis 1992;51(2):246–8.
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ACKNOWLEDGEMENTS 22. Schreiber A, et al. Membrane expression of proteinase 3 is genetically
determined. J Am Soc Nephrol 2003;14(1):68–75.
The authors thank Jana Vaskova for administrative and secretarial 23. Alberici F, Martorana D, Vaglio A. Genetic aspects of anti-neutrophil
support. cytoplasmic antibody-associated vasculitis. Nephrol Dial Transplant
2015;30(Suppl. 1):i37–45.
24. Kamesh L, et al. CT60 and +49 polymorphisms of CTLA 4 are associated
Please check your eBook at https://expertconsult.inkling.com/ with ANCA-positive small vessel vasculitis. Rheumatology (Oxford)
for self-assessment questions. See inside cover for registration 2009;48(12):1502–5.
details. 25. Langford CA, et al. An open-label trial of abatacept (CTLA4-IG) in
non-severe relapsing granulomatosis with polyangiitis (Wegener’s). Ann
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