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All-cause and cause-specific mortality risks in granulomatosis with polyangiitis Tan, Ju Ann


Background Granulomatosis with polyangiitis (GPA) is a form of ANCA-associated vasculitis (AAV), a heterogenous group of small vessel vasculitides associated with anti-neutrophil cytoplasmic antibodies (ANCA). There is still significant disease mortality despite advances in treatment of GPA. Furthermore, longitudinal data on secular trends in GPA mortality are scarce and most are from selected populations. We aim to determine all-cause and cause-specific mortality risks in GPA patients compared to the general population, as well as to estimate the difference in mortality risks in GPA patients between 1997-2004 and 2005-2012. Methods Chapter 2 was a systematic review and meta-analysis of observational studies in GPA. An extensive literature search was performed on the EMbase and Medline databases. Data was extracted for a pooled meta-analysis of standardized mortality ratios (SMR). Heterogeneity between studies was assessed using I2. Chapter 3 was a matched cohort population-based study using an administrative health database, comparing incident GPA cases and non-GPA individuals randomly selected from the general population. Primary outcome was death during the follow up period, 1997-2012. Cohorts were subdivided to early cohort (1997-2004) and late cohort (2005-2012). Hazard ratios (HR) were estimated using Cox proportional hazard regression models. Results From the meta-analysis, we found a 2.7-fold increased risk of death in AAV patients when compared to the general population. Subgroup analyses showed that mortality risks were higher in older cohorts with a trend towards improvement over time (i.e., midpoint of enrolment periods 1980-1993 and 1994-1999, vs. 2000-2005). From the matched cohort study, 370 GPA patients and 3,700 non-GPA individuals were included, with 68 and 310 observed deaths, respectively. Overall, the age, sex and entry time-adjusted all-cause mortality HR in the GPA cohort was 3.12 (95%CI 2.35-4.14). There was excess mortality from CVD causes, but not cancer, in the GPA cohort. All-cause mortality significantly improved between the early and late GPA cohorts (HR 5.61 vs. 2.33, respectively; p = 0.017). Conclusion There was a 3-fold increase in all-cause mortality risks in GPA patients, with excess mortality from CVD causes. There was a significant improvement in all-cause mortality risks over time but remained elevated compared to the general population.

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