Background/Purpose
Current data on mortality rates of primary vasculitis, which were tradionally associated with a dreadful prognosis, are limited. Therefore, we aimed to estimate the mortality rates of the main primary vasculitis disorders using the most current publicly available mortality data in the USA.
Methods
To obtain mortality rates of vasculitis as the underlying cause of death, we used the CDC Wonder Underlying Cause of Death database and its query system, which contains data from 1999 to 2010. We used the following ICD-10 codes for the queries: D69.0 (Allergic purpura) for Henoch-Schonlein purpura, D89.1 (Cryoglobulinaemia), M30.0 (Polyarteritis nodosa), M30.1 (Polyarteritis with lung involvement [Churg-Strauss]) for eosinophilic granulomatosis with polyangiitis, M30.3 (Mucocutaneous lymph node syndrome [Kawasaki]), M31.0 (Hypersensitivity angiitis) for Goodpasture’s syndrome, M31.3 (Wegener’s granulomatosis) for granulomatosis with polyangiitis (GPA), M31.4 (Aortic arch syndrome [Takayasu]), M31.5 (Giant cell arteritis [GCA] with polymyalgia rheumatica [PMR]) and M31.6 (Other giant cell arteritis) for GCA, M31.7 (Microscopic polyangiitis), and M35.2 (Behcet’s disease). Results were obtained by year, gender, and race. To obtain age-adjusted mortality rates we used year 2000 U.S. standard population. Mortality rates are given as number of deaths per million. Mantel-Haenszel chi-square was used to analyze trends.
Results
During the twelve-year period, vasculitis was the underlying cause of death of 7,888 patients. Age-adjusted mortality rate was 2.22 per million (95% CI: 2.17-2.27). There were more deaths in females (4,412) than in males (3,476), but the age-adjusted mortality rate was higher in males than in females (2.28 vs 2.16 per million). Age-adjusted mortality rate was clearly higher in Whites (2.34, 2.28-2.39) than in Black/African American population (1.19, 1.08-1.31). Regarding disease-specific mortality, GPA accounted for 51.2% of all vasculitis deaths. Interestingly, there were no deaths for GCA with PMR. Year 2000 showed the highest mortality (2.58, 2.40-2.77), whereas the lowest level was seen in 2008 (1.79, 1.64-1.94). Since 1999, there has been a significant trend to the decrease (p<0.0001).
Conclusion
The most current public data indicates that mortality by vasculitis remains very low, which is clearly related to the low incidence of these disorders. Age-adjusted mortality rate was higher in males and in White, which can be explained by the fact that GPA, which is more frequent in males and White, is responsible for half of the overall number of deaths. There is a clear trend to a progressive decrease of the mortality rates by primary vasculitis over the 12-year period of the study. Recent introduction of biological treatments, less toxic therapeutic regimens, such as shortening cyclophosphamide treatment, earlier diagnosis as a result of higher awareness and improved diagnostic tools may have presumably contributed to this decrease in mortality rates. Our findings should be taken with caution until quality studies to determine the reliability of the data about these rare diseases available in national databases are performed.
Disclosure:
A. Rodriguez-Pla,
None;
P. A. Monach,
None;
J. Rossello-Urgell,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/vasculitis-as-underlying-cause-of-death-in-the-united-states-1999-2010/