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.

Department of Clinical Sciences Section of Rheumatology Lund University Lund Sweden

Department of Medicine University of Cambridge Cambridge UK

Department of Nephrology 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic

Department of Nephrology and Dialysis Dubrava University Hospital Zagreb Croatia

Department of Nephrology and Medical and Health Sciences Linköping University Linköping Sweden

Department of Nephrology Saint Olavs University Hospital Trondheim Norway

Department of Rheumatology and Clinical and Experimental Medicine Linköping University Linköping Sweden

Division of Nephrology and Hypertension Department of Medicine UNC Kidney Center Chapel Hill NC USA

Division of Nephrology Department of Medicine Johns Hopkins University Baltimore MD USA

Emergency Care Clinic Haukeland University Hospital Bergen Norway

Faculty of Medicine Norwegian University of Science and Technology Trondheim Norway

Glasgow Renal and Transplant Unit Queen Elizabeth University Hospital Glasgow UK

Nephrology Dialysis and Transplantation Unit University of Bari Bari Italy

Renal Research Group Department of Clinical Medicine University of Bergen Bergen Norway

University of North Carolina School of Medicine Chapel Hill NC USA

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