Primary pulmonary alveolar proteinosis

Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about...

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Main Authors: Šarac Sanja, Milić Rade, Zolotarevski Lidija, Aćimović Slobodan, Tomić Ilija, Plavec Goran
Format: Article
Language:English
Published: Military Health Department, Ministry of Defance, Serbia 2012-01-01
Series:Vojnosanitetski Pregled
Subjects:
Online Access:http://www.doiserbia.nb.rs/img/doi/0042-8450/2012/0042-84501211005S.pdf
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author Šarac Sanja
Milić Rade
Zolotarevski Lidija
Aćimović Slobodan
Tomić Ilija
Plavec Goran
author_facet Šarac Sanja
Milić Rade
Zolotarevski Lidija
Aćimović Slobodan
Tomić Ilija
Plavec Goran
author_sort Šarac Sanja
collection DOAJ
description Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes whole-lung lavage, application of granulocyte-macrophage colonystimulating factor and lung transplantation. Case report. We reported a 51 year-old man with primary form of the disease. It was the second case of this extremely rare disease in the past 30 years in our clinic. The symptoms were longlasting dry cough, fever and physical deterioration. Chest Xray revealed bilateral pulmonary infiltrates; computed tomography showed patchy ground-glass opacification with interlobular thickening. The diagnosis was established by open lung biopsy. Additional tests were performed to exclude secondary form of the disease. Conclusion. We presented a rare clinical entity with typical clinical features and clinical and radiological course of the disease, in order to improve differential diagnostic approach to patients with bilateral lung infiltrations. In patients with pulmonary alveolar proteinosis timely diagnosis and adequate treatment can improve a prognosis.
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spelling doaj.art-f8a4f019cea242c2ba4f909ce755ebee2022-12-21T18:36:56ZengMilitary Health Department, Ministry of Defance, SerbiaVojnosanitetski Pregled0042-84502012-01-0169111005100810.2298/VSP1211005SPrimary pulmonary alveolar proteinosisŠarac SanjaMilić RadeZolotarevski LidijaAćimović SlobodanTomić IlijaPlavec GoranIntroduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes whole-lung lavage, application of granulocyte-macrophage colonystimulating factor and lung transplantation. Case report. We reported a 51 year-old man with primary form of the disease. It was the second case of this extremely rare disease in the past 30 years in our clinic. The symptoms were longlasting dry cough, fever and physical deterioration. Chest Xray revealed bilateral pulmonary infiltrates; computed tomography showed patchy ground-glass opacification with interlobular thickening. The diagnosis was established by open lung biopsy. Additional tests were performed to exclude secondary form of the disease. Conclusion. We presented a rare clinical entity with typical clinical features and clinical and radiological course of the disease, in order to improve differential diagnostic approach to patients with bilateral lung infiltrations. In patients with pulmonary alveolar proteinosis timely diagnosis and adequate treatment can improve a prognosis.http://www.doiserbia.nb.rs/img/doi/0042-8450/2012/0042-84501211005S.pdfpulmonary alveolar proteinosisdiagnosis, differentialradiographytomography, x-ray computedbiopsy
spellingShingle Šarac Sanja
Milić Rade
Zolotarevski Lidija
Aćimović Slobodan
Tomić Ilija
Plavec Goran
Primary pulmonary alveolar proteinosis
Vojnosanitetski Pregled
pulmonary alveolar proteinosis
diagnosis, differential
radiography
tomography, x-ray computed
biopsy
title Primary pulmonary alveolar proteinosis
title_full Primary pulmonary alveolar proteinosis
title_fullStr Primary pulmonary alveolar proteinosis
title_full_unstemmed Primary pulmonary alveolar proteinosis
title_short Primary pulmonary alveolar proteinosis
title_sort primary pulmonary alveolar proteinosis
topic pulmonary alveolar proteinosis
diagnosis, differential
radiography
tomography, x-ray computed
biopsy
url http://www.doiserbia.nb.rs/img/doi/0042-8450/2012/0042-84501211005S.pdf
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AT zolotarevskilidija primarypulmonaryalveolarproteinosis
AT acimovicslobodan primarypulmonaryalveolarproteinosis
AT tomicilija primarypulmonaryalveolarproteinosis
AT plavecgoran primarypulmonaryalveolarproteinosis