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Mortality has Decreased Significantly in GPA, but is it Good Enough?

November 9 2015 1:13 PM ET via RheumReports RheumReports

In the last two decades there have been several advancements in the management of granulomatosis with polyangiitis (GPA). The widespread use of anti-neutrophilic cytoplasmic antibodies (ANCA) has allowed for earlier diagnosis and the introduction of induction therapy followed by maintenance therapy, which decreased the incidence of adverse events. Newer pharmaceutical agents, and better management of comorbidities, may also improve outcomes. The impact of these advances on the long-term outcomes of patients with GPA was indirectly assessed by several studies using administrative databases, and presented at today's vasculitis sessions.

Using a general practice database with > 7 million subjects from the United Kingdom (abstract #1084), researchers found that that the overall mortality of GPA patients decreased significantly over time and that the rate of decrease was significantly higher than the mortality reduction seen in the general population. Nevertheless, the mortality rate in GPA was at least twice as high as age- and sex-matched subjects and 6 times higher in the first year after diagnosis.

Results from another administrative database from the USA (abstract #1083) revealed that rates of hospitalization in GPA patients have increased dramatically in recent years, both directly related to GPA or for other associated causes. The main reasons for hospital admission were cardiovascular (21%) and infection (17%), which were also the main causes of death. The good news is that even though rates of hospitalization have increased, mortality in hospitalized GPA patients has significantly improved and is approaching similar rates as non-GPA patients. We are obviously doing something right, but there is still room for improvement, particularly in decreasing complications of the disease and its therapies.

One of the major limitations with these large databases is that the diagnosis was not validated; therefore, misclassification is possible. New ways of validating diagnosis of GPA in administrative databases (abstract # 853) will hopefully improve our understanding of outcomes in this rare disease.


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About the Author

Dr. Lillian Barra
Dr. Lillian Barra

Lillian Barra is an Assistant Professor at Western University. Her research interests include autoimmune vascular disease and the role of autoantibodies in rheumatoid arthritis.

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