Differences in the immunolocalization of surfactant protein (SP)-A, SP-D, and KL-6 in pulmonary alveolar proteinosis.
Idiopathic pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by excessive accumulation of surfactant lipoprotein in alveoli, which is caused by autoantibody against granulocyte-macrophage colony-stimulating factor. The case of a 42-year-old man with idiopathic PAP, who had worked in steel and cement plants for the past 10 years, is presented. His serum anti-GM-CSF antibody level was markedly increased. Lung specimens obtained during video-assisted thoracoscopic surgery were examined on immunohistochemistry using mAb for localization of surfactant proteins A and D (SP-A and SP-D) and a mucin-like protein, KL-6. Furthermore, western blot analysis of his bronchoalveolar lavage fluid (BALF) was performed using anti-SP-A and anti-SP-D mAb. As well as KL-6, SP-A was localized in the intra-alveolar fine granular substances. But on HE staining the SP-D was localized in SP-A-negative foci corresponding to eosinophilic large globules that were surrounded by an SP-A-positive fine granular structure. On western blot the specificity of mAb was shown. In conclusion, this is the first report demonstrating the striking difference in the distribution of SP-A and SP-D in the intra-alveolar substance of a patient with idiopathic PAP.
Kobayashi
Makoto
M
Department of Respiratory Medicine, Hosogi Hospital, Kochi, Japan. kmakoto@jinseikai-group.or.jp
Takeuchi
Tamotsu
T
Ohtsuki
Yuji
Y
D000328:Adult; D015153:Blotting, Western; D019085:Fluorescent Antibody Technique, Direct; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000097:blood / Q000276:immunology; D006801:Humans; D007124:Immunoenzyme Techniques; D008168:Lung / Q000378:metabolism / Q000473:pathology; D008297:Male; D018396:Mucin-1 / Q000378:metabolism*; D011649:Pulmonary Alveolar Proteinosis / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D011650:Pulmonary Alveoli / Q000378:metabolism / Q000473:pathology; D037662:Pulmonary Surfactant-Associated Protein A / Q000378:metabolism*; D037663:Pulmonary Surfactant-Associated Protein D / Q000378:metabolism*; D011663:Pulmonary Surfactants / Q000378:metabolism*; D013902:Radiography, Thoracic
C060192:MUC1 protein, human; D018396:Mucin-1; D037662:Pulmonary Surfactant-Associated Protein A; D037663:Pulmonary Surfactant-Associated Protein D; D011663:Pulmonary Surfactants; D016178:Granulocyte-Macrophage Colony-Stimulating Factor