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All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type

Zachary S Wallace1,2,3,4, Xiaoqing Fu1,2,3, Tyler Harkness1,2,3

  • 1Clinical Epidemiology Program, Mongan Institute.

Rheumatology (Oxford, England)
|December 18, 2019
PubMed

Insights

Premature mortality in ANCA-associated vasculitis is linked to cardiovascular disease, infection, and malignancy. Patients with MPO-ANCA have a higher risk of cardiovascular death compared to PR3-ANCA patients.

Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • ANCA-associated vasculitis (AAV) is a group of rare autoimmune diseases characterized by inflammation of small blood vessels.
  • Understanding the causes of premature mortality in AAV is crucial for improving patient outcomes.
  • Stratifying mortality by ANCA type (MPO-ANCA vs. PR3-ANCA) may reveal distinct risk profiles.

Purpose of the Study:

  • To investigate the causes of death in a contemporary cohort of patients diagnosed with ANCA-associated vasculitis.
  • To analyze mortality patterns based on the specific type of anti-neutrophil cytoplasmic antibody (ANCA) detected.

Main Methods:

  • A consecutive inception cohort of AAV patients diagnosed between 2002 and 2017 was identified.
  • Vital status was determined using the National Death Index, and mortality incidence was calculated.
  • Standardized mortality ratios (SMRs) were compared to the general population, and Cox regression analyzed MPO-ANCA vs. PR3-ANCA cases.

Main Results:

  • The cohort comprised 484 patients; 130 deaths occurred over 3385 person-years, resulting in an SMR of 2.3.
  • Cardiovascular disease (CVD), malignancy, and infection were the leading causes of death.
  • Infection posed the greatest excess mortality risk for both MPO-ANCA and PR3-ANCA patients, while MPO-ANCA patients showed a higher risk of CVD death.

Conclusions:

  • Premature mortality in AAV is primarily attributed to CVD, infection, malignancy, and renal disease.
  • Infection represents the most significant excess mortality risk, particularly in MPO-ANCA and PR3-ANCA patients.
  • MPO-ANCA patients face a substantially higher risk of cardiovascular death compared to PR3-ANCA patients.
Abstract

Keywords:
ANCA-associated vasculitiscardiovascular diseasecause of deathmortality

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