open access

Vol 83, No 6 (2015)
CASE REPORTS
Submitted: 2015-11-10
Accepted: 2015-11-10
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Atypical image of pulmonary alveolar proteinosis — a case report

Justyna Fijołek, Elżbieta Wiatr, Lucyna Opoka, Piotr Rudziński, Małgorzata Nierebińska, Małgorzata Szołkowska, Kazimierz Roszkowski-Śliż
DOI: 10.5603/PiAP.2015.0074
·
Pneumonol Alergol Pol 2015;83(6):453-456.

open access

Vol 83, No 6 (2015)
CASE REPORTS
Submitted: 2015-11-10
Accepted: 2015-11-10

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.

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.

Get Citation

Keywords

respiratory failure, pulmonary alveolar proteinosis, computed tomography, whole lung lavage

About this article
Title

Atypical image of pulmonary alveolar proteinosis — a case report

Journal

Advances in Respiratory Medicine

Issue

Vol 83, No 6 (2015)

Pages

453-456

DOI

10.5603/PiAP.2015.0074

Bibliographic record

Pneumonol Alergol Pol 2015;83(6):453-456.

Keywords

respiratory failure
pulmonary alveolar proteinosis
computed tomography
whole lung lavage

Authors

Justyna Fijołek
Elżbieta Wiatr
Lucyna Opoka
Piotr Rudziński
Małgorzata Nierebińska
Małgorzata Szołkowska
Kazimierz Roszkowski-Śliż

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