Nationwide Trends in Hospitalizations and In‐Hospital Mortality in Granulomatosis With Polyangiitis (Wegener's)

Abstract

Background Granulomatosis with polyangiitis (GPA) is a type of ANCA-associated vasculitis associated with severe end-organ damage and treatment-related complications that often lead to hospitalization and death. Nationwide trends in hospitalizations and in-hospital mortality over the past two decades are unknown and were evaluated in this study. Methods Using the National Inpatient Sample (NIS), the largest all-payer inpatient database in the US, the trends in hospitalizations with a discharge diagnosis of GPA (formerly Wegener’s granulomatosis; ICD-9-CM: 446.4) between 1993 and 2011 were studied. Analyses were performed using hospital-level sampling weights to obtain US national estimates. Results From 1993 to 2011, the annual hospitalization rate for patients with a principal diagnosis of GPA increased by 24% from 5.1 to 6.3/1,000,000 US persons (P-for-trend<0.0001); however, their in-hospital deaths declined by 73% from 9.1% to 2.5% (P-for-trend<0.0001), resulting in a net 66% reduction of the annual in-hospital mortality rate. The median length of stay declined by 20% from 6.9 days in 1993 to 5.5 days in 2011 (P-for-trend=0.0002). Infection was the most common principal discharge diagnosis when GPA was a secondary diagnosis, including among those who died during hospitalization. Conclusion The findings from these nationally representative, contemporary, inpatient data indicate that in-hospital mortality of GPA has declined substantially over the past two decades while the overall hospitalization rate for GPA increased slightly. Infection remains a common principal hospitalization diagnosis among GPA patients, including hospitalizations resulting in mortality.

The Epidemiology of Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis in Olmsted County, Minnesota

“…Interestingly, in our cohort, the survival rate for those with EGPA and those with MPA continued to decrease during the 10 years after diagnosis, in contrast to that in previous reports, in which higher mortality in AAV was attributed to the first year after diagnosis . This finding may be a reflection of the modern immunosuppressive strategies being utilized for AAV , leading to remission in more than 90% of cases and, thus, improved survival in the first year, although survivorship in EGPA and MPA is overall still lower than that in GPA.…”

“…Surprisingly, we observed that GPA‐related mortality was no longer increased when compared to the mortality rate in the general population . Reasons for this observation of improved survivorship in those with GPA are uncertain, but may have been influenced by improved case identification, as was shown by the increased incidence of GPA, and likely could have been influenced by the close and consistent multispecialty follow‐up care provided in a tertiary care center once the diagnosis was established.…”

Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression

“…We know that cardiac and non‐cardiac vascular disease is significantly more likely in the presence of extra articular RA . GPA is also associated with a risk of mortality, although more recent evidence shows that despite hospitalizations increasing for this condition, mortality has significantly reduced over the last 15 to 20 years, presumably due to improved immunosuppressive therapy . Mortality linked to RA has also significantly decreased over the last decade .…”

Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working‐Age Adult Populations In the United States

“…For example, a study of GPA patients in an all-payer U.S. inpatient database (National Inpatient Sample) reported that infections (particularly respiratory tract infections) were the most frequent reason for hospitalization (25%) and also a primary contributor to death (16.4% of all in-hospital mortality cases)(41). In our study, pediatric and working-age adult GPA cohorts had comparable high rates of severe infections, whereas the hospitalization rate was higher in pediatric GPA patients.…”