[Pulmonary alveolar proteinosis].
Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the accumulation of proteinaceous material within alveoli. In order to evaluate the clinical features and the course of PAP we reviewed 7 cases (2F/5M) diagnosed during a 11-year period (1989-1999). The mean age of patients was 40.7 +/- 11.2 years. Diagnosis was obtained by open lung biopsy in all cases. Clinical findings included dyspnea (43%), cough (28%) and crackles (28%). Lung function tests were normal in 5 cases and showed a moderate restrictive pattern in 1 and mild airflow obstruction in 1. Three patients had reduced Dlco (mean was 63% of predicted). Four patients had hypoxemia at rest. Chest X-ray revealed bilateral alveolar opacities (71%), involving perihilar areas and lower lobes. HRCT scans demonstrated diffuse ground glass opacities (83%) with interlobular septa thickening (50%). Three patients were treated with repeated segmental BAL (2 improved). The spontaneous partial remission occurred in 4.
I Kliniki Gruźlicy i Chorób Płuc Instytutu Gruźlicy, i Chorób Płuc w Warszawie.
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Sobiecka
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Korzeniewska-Koseła
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Kuś
D000328:Adult; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis*; D011856:Radiographic Image Enhancement; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed