GM-CSF Inhalation Promoting Organization

Pulmonary Alveolar Proteinosis

Incidence of autoimmune pulmonary alveolar proteinosis

The number of patients with autoimmune alveolar proteinosis in Japan is estimated to be 3,300. The incidence rate (number of new cases per million people per year) is 1.65 people/year.

Up to May 2020, Niigata University collected sera from patients with autoimmune pulmonary alveolar proteinosis from all over Japan to measure anti-GM-CSF autoantibody concentrations. From 2006 to 2016, the data from 702 patients with autoimmune pulmonary proteinosis, from different medical facilities, were collected at Niigata University.

We learned the following from this collection:

  • There is no difference in the incidence of patients from Hokkaido to Okinawa, indicating that differences in climate or soil have no impact.
  • Using Niigata Prefecture (population of approximately 2.28 million people ) as a model, the number of new patients each year (average [ 95% confidence interval ] ) is estimated to be 3.9 [1.5–10.2 people ]/year.
  • Since there are no regional differences, the number of new patients nationwide is estimated to be 211 [81–549] per year. Incidence rate (new cases per million people per year ) is 1.65 [0.64–4.31 ]/year.

Kitamura et al. ERJ Open Res 2019; 5: 00190-2018.

Location of medical facilities where patients were seen from 2006-2016. (Each dot represents one patient.)
 - Kitamura et al. ERJ Open Res 2019; 5: 00190-2018.
Correlation of population (x-axis) and number of patients (y-axis) by prefecture
 - Kitamura et al. ERJ Open Res 2019; 5: 00190-2018.