Originally published by 2 Minute Medicine® (view original article). Reused on AccessMedicine with permission.

1. In this study of decedents from 1999 to 2017, mortality due to antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) -related illness was found to have decreased by an average of 1.6% per year.

2. Following stratification, non-Hispanic whites, men, and Midwesterners appeared to be disproportionately affected by AAV, but it remained unclear whether this phenomenon should be attributed to biological or social factors.

Evidence Rating Level: 2 (Good)

Study Rundown:

Antineutrophil cytoplasmic autoantibody-associated vasculitides (AAVs) are small vessel diseases that can affect nearly every organ system and are associated with significant morbidity and mortality. Historically, patients have largely relied on immunosuppressive therapy, but the effectiveness of such treatment was unclear as long-term outcomes remained poorer than the general population. This study sought to identify mortality trends of AAV in the United States in the past two decades. Overall, the age-adjusted mortality rate was found to have decreased by an average of 1.6% per year. Data from multiple-cause-of-death records indicated that AAV remained the predominant underlying cause of death (UCOD), underscoring the importance of clinical awareness and continued research & development of novel therapies. The study boasts a large sample size. However, it is limited by potential misreporting of AAV on death certificates that could have skewed results. The absence of similar large-scale stratified studies makes it difficult to validate the findings as well.

In-Depth [retrospective cohort]:

For this study, multiple-cause-of-death records were drawn from the Centers for Disease Control and Prevention’s Wide-ranging Online Data for Epidemiologic Research Data- base from 1999 to 2017. AAV mortality was defined as decedents whose causes of death (primary or secondary) included granulomatosis with polyangiitis, microscopic polyangiitis, or eosinophilic granulomatosis with polyangiitis. Of these, granulomatosis with polyangiitis was the leading cause of death at 85%. In conjunction, these AAVs were by far the most common UCOD (64%), trailed by cardiovascular disease (12%), cancer (6%), and pulmonary disorder (5%). The average age-adjusted mortality rate over this period was 1.86 (95% CI, 1.83 to 1.90) per 1,000,000 persons, with non-Hispanic whites, men, and Midwesterners bearing the brunt. Overall mortality rate abated by an average of 1.6% (CI, 2.6% to 0.7%) per year.

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