HLA allele variation as a potential explanation for the geographical distribution of granulomatosis with polyangiitis

Richard A Watts, Alexander Macgregor, Sarah L Mackie

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26 Citations (Scopus)


Granulomatosis with polyangiitis (GPA) is a rare autoimmune systemic vasculitis considered to result from the interaction of environmental factors with a genetically predisposed host. The HLA-DPB1*0401 allele, the PI*Z allele of the gene encoding α1-antitrypsin (SERPINA1) and the proteinase 3 (PRTN3) gene have been associated with GPA. The incidence of GPA is lower in non-Caucasian populations and has been associated with higher latitude. Our aim was to determine whether variation in population carrier frequency of the HLA-DPB1*0401 and PI*Z alleles could explain in part the variation in GPA incidence between countries.
Original languageEnglish
Pages (from-to)359-362
Issue number2
Early online date28 Aug 2014
Publication statusPublished - 2015


  • granulomatosis with polyangiitis
  • epidemiology
  • genetics
  • HLA
  • vasculitis

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