Israel Medical Association Journal

 Background:  Pulmonary alveolar proteinosis is a rare disease in which a surfactant-like phospholipid-rich protein accumulates in the lungs. The disease is amenable to effective therapy by total lung lavage.       

Objectives: To investigate the prevalence, ethnic distribution and course of PAP  in Israel.

Methods: A countrywide survey was conducted during which pulmonologists were questioned about patients with PAP. The patients were examined and their charts, radiological images, pathological slides and physiological data were reviewed.

Results: The survey yielded 15 patients (8 females) during the period 1976–98 (14 in the last decade), giving a prevalence of 3.7x10⁶ and an incidence of 0.36x10⁶/year.

Mean age of the patients was 33±13 years (range 0.5–46 years). Seven patients were North African (two were siblings), four were 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 bronchoalveolar lavage treatments. The subjective and physiological response was favorable, but there was less consistent radiological improvement.

Conclusion: The prevalence of PAP in Israel is approximately 3.7x10⁶. Most 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 similar. This clustering suggests the existence of a genetic predisposition. The course of the disease appears to be similar to that reported elsewhere.

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PAP = pulmonary alveolar proteinosis

Methods: The demographic data and clinical manifestations of 144 TA¹ patients in this large multicenter study were recorded and compared with data obtained in a previous study.

Results: The patient population was older, with 24% ≥80 years compared to 6% in the previous study.  There was an increase in the number of nonspecific presenting symptoms, and less patients presented with the “classical” manifestations of headache (81% vs. 71%), fever (83% vs. 40%), jaw claudication (21% vs. 13%), and visual symptoms (47% vs. 24%). The median time from presentation to diagnosis was significantly reduced, from 5 to 1.5 months.

Conclusions: There were substantial changes in the clinical presentation of TA patients in Israel during 1980–95 compared to patients diagnosed prior to 1978. It is suggested that these changes may be attributed not only to the influence of aging of the population, but are due largely to increasing physician awareness to the spectrum of manifestations of TA, which leads to earlier diagnosis.

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¹TA = temporal arteritis