title: Atypical image of pulmonary alveolar proteinosis - a case report.
abstract: Pulmonary alveolar proteinosis is a very rare interstitial lung disease caused by abnormal intra-alveolar surfactant accumulation. Usually, it appears as a "crazy-paving" pattern on high-resolution computed tomography. The image is so typical, that together with the characteristic bronchoalveolar lavage examination with presence of Periodic Acid Schiff positive substance is sufficient for establishing diagnosis, without histological confirmation. We present the case of the young woman with severe dyspnoea suspected of acute hypersensitivity pneumonia. The computed tomography showed numerous intralobular nodules uniformly distributed troughout the lungs. Treatment by corticosteroids had no clinical effect and next computed tomography showed progression. Despite the high risk of complications (patient had a respiratory failure), a surgical lung biopsy was performed and the histopathological diagnosis of pulmonary alveolar proteinosis was made. The whole lung lavage procedure performed twice caused regression of radiological lesions and respiratory failure.
authors.affiliation: The Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases, Warsaw, Poland. jfijolek@op.pl.
authors.forename: Justyna
authors.initials: J
authors.lastname: Fijołek
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Wiatr
authors.forename: Lucyna
authors.initials: L
authors.lastname: Opoka
authors.forename: Piotr
authors.initials: P
authors.lastname: Rudziński
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Nierebińska
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Szołkowska
authors.forename: Kazimierz
authors.initials: K
authors.lastname: Roszkowski-Śliż
mesh_terms: D000328:Adult; D018893:Bronchoalveolar Lavage / Q000379:methods; D003371:Cough / Q000209:etiology; D005260:Female; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008168:Lung / Q000000981:diagnostic imaging; D016676:Macrophages, Alveolar / Q000473:pathology*; D011649:Pulmonary Alveolar Proteinosis / Q000000981:diagnostic imaging* / Q000628:therapy*; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed
keywords: computed tomography; pulmonary alveolar proteinosis; respiratory failure; whole lung lavage