Background: Pulmonary alveolar proteinosis is a rare disease in which a sur
factant-like phospholipid-rich protein accumulates in the lungs. The diseas
e is amenable to effective therapy by total lung lavage. Objectives: To inv
estigate the prevalence, ethnic distribution and course of PAP(1) in Israel
Methods: A countrywide survey was conducted during which pulmonologists wer
e questioned about patients with PAP. The patients were examined and their
charts, radiological images, pathological slides and physiological data wer
Results: The survey yielded 15 patients (8 females) during the period 1976-
98 (14 in the last decade), giving a prevalence of 3.7x10(6) and an inciden
ce of 0.36x10(6)/year. Mean age of the patients was 33+/-13 years (range 0.
5-46 years). Seven patients were North African (two were siblings), four we
re from Iraq and two were Arabs; there was only one Ashkenazi Jew (a child)
. Symptoms at the onset were dyspnea and chest pain. Spontaneous remission
occurred in at least 3 patients, and 10 patients required 1-4 bronchoalveol
ar lavage treatments. The subjective and physiological response was favorab
le, but there was less consistent radiological improvement.
Conclusion: The prevalence of PAP in Israel is approximately 3.7x10(6). Mos
t cases occurred in Jews who had immigrated from North Africa or Iraq, and
two were siblings. The prevalence among the Arab population appears to be s
imilar. This clustering suggests the existence of a genetic predisposition.
The course of the disease appears to be similar to that reported elsewhere