Proteinase-3 and myeloperoxidase serotype in relation to demographic factors and geographic distribution in anti-neutrophil cytoplasmic antibody-associated glomerulonephritis

Research output: Contribution to journalArticle


Background: In anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis, antigen specificity varies between myeloperoxidase (MPO) and proteinase 3 (PR3). This has been reported to vary in relation to age, gender, geography and extrarenal manifestations. However, studies are difficult to compare as criteria for inclusion vary. The aim of this study was to investigate the relationship between ANCA serotype, latitude, ultraviolet (UV) radiation levels, age, gender and renal function at diagnosis in a large study with uniform inclusion criteria. Methods: Patients with biopsy-proven ANCA-associated glomerulonephritis were identified from regional or nationwide registries in 14 centres in Norway, Sweden, the UK, the Czech Republic, Croatia, Italy and the USA during the period 2000-13. UV radiation levels for 2000-13 in Europe were obtained from the Swedish Meteorological and Hydrological Institute. Results: A total of 1408 patients (45.2% PR3-ANCA) were included in the study. In univariable analysis, PR3-ANCA was significantly associated with male gender {odds ratio [OR] 2.12 [95% confidence interval (CI) 1.71-2.62]}, younger age [OR per year 0.97 (95% CI 0.96-0.98)] and higher glomerular filtration rate [OR per mL/min 1.01 (95% CI 1.01-1.02); P < 0.001] at diagnosis but not with latitude or UV radiation. In multivariable logistic regression analysis, latitude and UV radiation also became significant, with higher odds for PR3-ANCA positivity at northern latitudes/lower UV radiation levels. However, the latitudinal difference in MPO:PR3 ratio is smaller than differences previously reported concerning microscopic polyangiitis and granulomatosis with polyangiitis. Conclusions: The ratio between PR3-ANCA and MPO-ANCA varies in glomerulonephritis with respect to age, gender, renal function and geographic latitude/UV radiation levels.


  • Maria Weiner
  • Rune Bjørneklett
  • Zdenka Hrušková
  • Bruce Mackinnon
  • Caroline J. Poulton
  • Leo Sindelar
  • Aladdin J. Mohammad
  • Per Eriksson
  • Loreto Gesualdo
  • Duvuru Geetha
  • Matija Crnogorac
  • David Jayne
  • Susan L. Hogan
  • Colin Geddes
  • Vladimir Tesar
  • Knut Aasarød
  • Mårten Segelmark
External organisations
  • Linköping University
  • University of Bergen
  • Haukeland University Hospital
  • General University Hospital in Prague
  • University of North Carolina
  • University of Cambridge
  • University of Bari Aldo Moro
  • Johns Hopkins University
  • St. Olav’s University Hospital
  • Norwegian University of Science and Technology
  • Charles University in Prague
  • Queen Elizabeth University Hospital
  • Dubrava University Hospital
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Urology and Nephrology
  • Public Health, Global Health, Social Medicine and Epidemiology
Original languageEnglish
Pages (from-to)301-308
Number of pages8
JournalNephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
Issue number2
Publication statusPublished - 2019
Publication categoryResearch