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Abstract: SA-PO639

Improved Survival due to Better Renal Outcomes in Danish Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (AAV) During the Years 2000-2015: A Nationwide Study

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Nelveg-Kristensen, Karl Emil, Rigshospitalet, Copenhagen, Denmark, Cambridge, United Kingdom
  • Szpirt, Wladimir M., Rigshospitalet P2132, Copenhagen, Denmark
  • Carlson, Nicholas, Rigshospitalet, Copenhagen, Denmark, Cambridge, United Kingdom
  • Dieperink, Hans Herman, Odense University hospital, Odense, Denmark
  • Krarup, Elizabeth, Herlev Hospital, 2730 Herlev, Denmark
  • Ivarsen, Per R., Aarhus University Hospital, Aarhus, Denmark
  • Gregersen, Jon Waarst, Aalborg University Hospital, Aalborg, Denmark
  • Torp-Pedersen, Christian, Aalborg University Hospital, Aalborg, Denmark
  • Egfjord, Martin, Rigshospitalet, Copenhagen, Denmark
Background

AAV (Granulomatosis with polyangiitis and microscopic polyangiitis) carries significant risk of morbidity and mortality, notwithstanding adequate treatment. Contemporary large-scale descriptive studies have been challenged by the rare occurrence of these diseases. Accordingly, by use of Danish nationwide healthcare registries, we examined the temporal progression of incidence and prognosis of AAV during 2000-2015.

Methods

All patients with incident AAV, regardless of primary organ manifestation were included by use of ICD10 diagnostic codes (positive predictive value of > 90%) and grouped in five-year intervals (P1: 2000-2004, P2: 2005-2009, P3: 2010-1015). Absolute risk ratios (ARR) adjusted for age, sex and advanced disease severity (>10 days of initial hospital stay), as well as cumulative incidences were computed in R version 3.5.0.

Results

We identified 1634 patients (52% male), corresponding to an overall incidence of 18.2 persons/million/year (P1: 12.1; P2: 16.3; P3: 21.0), and 425 (26% [P1: 34.6%; P2: 28.5%; P3: 19.4%]) met the criteria of advanced disease severity. Mean age was 60.3 (IQR 21.0) years and mean follow-up was 5.9 (IQR 4.0) years. 571 (34.9%) patients died (uncensored 5-year mortality of 20.3%) resulting in an ARR for P2 and P3 as compared to P1 of 0.80 (CI 0.65-0.97, P=0.028), and 0.40 (CI 0.30-0.51, P<0.001). 274 patients developed end-stage renal disease (16.8 % [P1: 23.3%; P2: 17.6%; P3: 12.5%]), similarly with ARR decreasing over time: P2 0.62 (CI 0.43-0.89, P=0.009) and P3 0.54 (CI 0.37-0.78, P=0.001) relative to P1. The overall risk of death associated with need of dialysis or chronic kidney involvement within 30 days of discharge as compared to no renal involvement was 1.81 (CI 1.41-2.33, P<0.001) and 1.39 (CI 1.11-1.76, P=0.005). During follow-up 526 (32.2% [P1: 39.6%; P2: 30.4%; P3: 29.3%]) patients developed chronic kidney disease.

Conclusion

AAV remain a group of diseases associated with high morbidity and mortality; however, although incidence of AAV is increasing, the absolute risk of death appears to be declining, putatively in part due to earlier detection of incident episodes and better renal outcomes.