Is granulomatosis with polyangiitis in Asia different from the West?

Int J Rheum Dis. 2019 Jan:22 Suppl 1:90-94. doi: 10.1111/1756-185X.13398. Epub 2018 Oct 18.


The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries. The annual incidence of GPA among Asians varies between 0.37/million to 2.1/million population. The prevalence of GPA has been estimated to be 1.94/100 000 in a Chinese population. Polymorphisms in class II major histocompatibility genes and ETS1 proto-oncogene has been shown in Asian patients with GPA. There is a difference in mean age at onset and proteinase 3 (PR3) or myeloperoxidase (MPO) positivity in GPA patients from different Asian countries. Those from India had mean age of 40 years and those from Japan had mean age of 65 years. Sixty percent of GPA patients from China and Japan were MPO ANCA positive while the majority of patients from India and Korea were PR3 positive. Geographical variation with lower frequency of renal involvement in Indian studies and higher frequency in Chinese patients has also been noted. Treatment outcomes have been similar to those reported from other parts of the world. Remission was achieved in about two-thirds of patients while relapses were noted in one-third to half of the patients. Apart from minor differences in the organ systems involved, MPO-ANCA GPA and PR3-ANCA GPA had similar rates of remission and relapses.

Keywords: Asia; granulomatosis with polyangiitis; vasculitis.

Publication types

  • Review

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Asia / epidemiology
  • Asian People* / genetics
  • Female
  • Genetic Predisposition to Disease
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / ethnology*
  • Granulomatosis with Polyangiitis / genetics
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Phenotype
  • Polymorphism, Genetic
  • Prevalence
  • Prognosis
  • Proto-Oncogene Mas
  • Risk Factors
  • White People* / genetics