Abstract
Background: Goodpasture’s syndrome is a rare, life-threatening, small vessel vasculitis. Given its rarity, data on its inpatient burden and resource utilization are lacking. We conducted this study aiming to assess inpatient prevalence, mortality, and resource utilization of Goodpasture’s syndrome in the United States. Methods: The 2003–2014 National Inpatient Sample was used to identify patients with a principal diagnosis of Goodpasture’s syndrome. The inpatient prevalence, clinical characteristics, in-hospital treatment, end-organ failure, mortality, length of hospital stay, and hospitalization cost were studied. Multivariable logistic regression was performed to identify independent factors associated with in-hospital mortality. Results: A total of 964 patients were admitted in hospital with Goodpasture’s syndrome as the principal diagnosis, accounting for an overall inpatient prevalence of Goodpasture’s syndrome among hospitalized patients in the United States of 10.3 cases per 1,000,000 admissions. The mean age of patients was 54 ± 21 years, and 47% were female; 52% required renal replacement therapy, whereas 39% received plasmapheresis during hospitalization. Furthermore, 78% had end-organ failure, with renal failure and respiratory failure being the two most common end-organ failures. The in-hospital mortality rate was 7.7 per 100 admissions. The factors associated with increased in-hospital mortality were age older than 70 years, sepsis, the development of respiratory failure, circulatory failure, renal failure, and liver failure, whereas the factors associated with decreased in-hospital mortality were more recent year of hospitalization and the use of therapeutic plasmapheresis. The median length of hospital stay was 10 days. The median hospitalization cost was $75,831. Conclusion: The inpatient prevalence of Goodpasture’s syndrome in the United States is 10.3 cases per 1,000,000 admissions. Hospitalization of patients with Goodpasture’s syndrome was associated with high hospital inpatient utilization and costs.
Highlights
Goodpasture’s syndrome (GS) is a rare, life-threatening, small vessel vasculitis that is mediated by circulating anti-glomerular basement membrane autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen, targeting capillaries of the kidneys and lungs [1,2,3,4]
It is considered as one of the organ-specific autoimmune diseases that typically presents as a rapidly progressive glomerulonephritis (RPGN), accompanied by alveolar hemorrhage with pathology characterized by crescentic glomerulonephritis with classic linear polyclonal immunoglobulin (Ig) G deposits on immunofluorescence staining of the GBM on analysis of kidney biopsy samples [1,2,5,6,7]
The factors associated with increased in-hospital mortality were age older than 70 years, sepsis, the development of respiratory failure, circulatory failure, renal failure, and liver failure, whereas the factors associated with decreased in-hospital mortality were more recent year of hospitalization and the use of therapeutic plasmapheresis
Summary
Goodpasture’s syndrome (GS) is a rare, life-threatening, small vessel vasculitis that is mediated by circulating anti-glomerular basement membrane (anti-GBM) autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen, targeting capillaries of the kidneys and lungs [1,2,3,4]. We conducted this study aiming to assess inpatient prevalence, mortality, and resource utilization of Goodpasture’s syndrome in the United States. Methods: The 2003–2014 National Inpatient Sample was used to identify patients with a principal diagnosis of Goodpasture’s syndrome. Hospitalization of patients with Goodpasture’s syndrome was associated with high hospital inpatient utilization and costs
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