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Differences in the immunolocalization of surfactant protein (SP)‐A, SP‐D, and KL‐6 in pulmonary alveolar proteinosis
Author(s) -
Kobayashi Makoto,
Takeuchi Tamotsu,
Ohtsuki Yuji
Publication year - 2008
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.2007.02212.x
Subject(s) - pulmonary alveolar proteinosis , pathology , bronchoalveolar lavage , pulmonary surfactant , surfactant protein a , immunohistochemistry , surfactant protein d , western blot , lung , eosinophilic , mucin , lamellar granule , muc1 , chemistry , antibody , medicine , microbiology and biotechnology , biology , immunology , immune system , ultrastructure , biochemistry , innate immune system , gene
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.