Yeungnam Univ J Med.  2010 Jun;27(1):57-62. 10.12701/yujm.2010.27.1.57.

A Case of Pulmonary Alveolar Proteinosis

• Woo DH
• Park JE
• Ryu YH
• Kim HJ
• Shin KC
• Chung JH
• Lee KH

• KMID: 1462713
• DOI: http://doi.org/10.12701/yujm.2010.27.1.57

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disorder that's characterized by accumulation of surfactant components in the alveolar space. Idiopathic PAP is recognized as an autoimmune disease that's due to impaired alveolar macrophage function and this caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). We report here a case of pulmonary alveolar proteinosis that was deemed interstitial lung disease at the initial diagnosis. A 61-year-old man presented with intermittent blood tinged sputum and dyspnea on exertion. The man was a painter for 30 years and he had a 10 pack-years smoking history. Chest computerized tomography (CT) revealed multifocal ground-glass opacity with interstitial thickening at both lungs. His pulmonary function tests and methacholine test revealed non specific results. He was diagnosed with interstitial lung disease on the basis of the chest CT finding and occupational history. However, seven months later, his symptoms progressed. Follow-up chest CT was performed. Wedge resection via video-assisted thoracoscopic surgery (the anterior basal segment of the left lower lobe) was done. Microscopic examination showed large groups of alveoli with excessive amounts of surfactant and a complex mixture of protein and lipid (fat) molecules. Finally, he was diagnosed as having pulmonary alveolar proteinosis.

Keyword

Pulmonary alveolar proteinosis; Chest CT

MeSH Terms

Autoantibodies
Autoimmune Diseases
Benzeneacetamides
Dyspnea
Follow-Up Studies
Granulocyte-Macrophage Colony-Stimulating Factor
Humans
Lung
Lung Diseases, Interstitial
Macrophages, Alveolar
Methacholine Chloride
Middle Aged
Piperidones
Pulmonary Alveolar Proteinosis
Respiratory Function Tests
Smoke
Smoking
Sputum
Thoracic Surgery, Video-Assisted
Thorax
Tolnaftate
Autoantibodies
Benzeneacetamides
Granulocyte-Macrophage Colony-Stimulating Factor
Methacholine Chloride
Piperidones
Smoke
Tolnaftate