New insights on rare pulmonary alveolar proteinosis
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Among adults with pulmonary alveolar proteinosis admitted to U.S. hospitals over a 3-year period, the inpatient mortality rate was 5% and the overall annual cost burden was $5 million, researchers reported at the virtual CHEST Annual Meeting.
“[Pulmonary alveolar proteinosis] is a rare disease. ... Some pulmonologists probably won’t see it in their professional lives,” Rodrigo Garcia-Tome, MD, with the University of Southern California, Los Angeles, said during his presentation.
Pulmonary alveolar proteinosis was first described in 1958. There is a paucity of large-scale, multicenter, epidemiologic studies on pulmonary alveolar proteinosis due to the rarity of the disorder, he said.
Garcia-Tome and colleagues analyzed the National Inpatient Sample database and identified 500 patients (mean age, 41 years; 58% white; 55% men) who were admitted to a U.S. hospital with a primary diagnosis of pulmonary alveolar proteinosis from January 2012 to December 2014. The researchers assessed frequency of admission, interventions performed, in-hospital mortality rate, length of stay and hospitalization costs
Most patients were admitted to large tertiary hospital centers. There was no significant difference in median annual income. Most patients were insured with private insurance or Medicare.
In 2013, there was an 8.6% mortality rate and a 6.5% mortality rate for 2014. No mortality was observed in 2012. Of 500 patients, 25 patients died resulting in a 5% overall mortality rate during the study period.
Over the 3-year period, 195 whole lung lavage procedures were performed among the 500 patients with pulmonary alveolar proteinosis. Mean length of stay was 6.2 days.
Twenty-five patients died in hospital during the study period, representing a 5% in-hospital mortality rate, Garcia-Tome said.
The mean charge of admission was $100,257 (95% CI, 51,345-149,168) and mean actual cost per admission was $29,932 (95% CI, 13,739-46,124), according to the results.
“Pulmonary alveolar proteinosis affects slightly more men than women. It is primary occurring in Caucasians and Blacks, it affects every socioeconomic status, has an average inpatient mortality of about 5%, the cost of admission is around $30,000 and it is usually managed in a tertiary center, which had more expertise with this disease,” Garcia-Tome said.
Editor’s note: This article was updated on Oct. 23, 2020, to correctly note the authors’ affiliation. We regret the error.