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Title of Journal: Lung

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Abbravation: Lung

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Springer-Verlag

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10.1007/978-3-642-16607-5_30

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1432-1750

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Idiopathic Pulmonary Fibrosis is Associated with C

Authors: Ahmed Fahim Mei C Chong Michael G Crooks Simon P Hart
Publish Date: 2012/05/16
Volume: 190, Issue: 4, Pages: 451-458
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Abstract

Idiopathic pulmonary fibrosis IPF is a restrictive fibrotic lung disease of uncertain etiology Alveolar epithelial injury may be one of the inciting triggers in the pathogenesis of this disorder We hypothesized that circulating antibodies to alveolar epithelial and endothelial cells may be involved in the pathogenesis of IPFAntibodies to alveolar epithelial and endothelial cells were analyzed by indirect immunofluorescence using alveolar epithelial cells A549 and human umbilical vein endothelial cells respectively IgG and IgM antibodies in patients’ serum were evaluated Patterns of immunofluorescence including membranous cytoplasmic and nuclear staining were analyzed by fluorescence microscopy The severity of immunofluorescence was divided into mild moderate and severe categories Fiftysix patients IPF = 28 nonIPF ILD = 9 nonILD control = 19 were evaluated for antiepithelial antibodies and 28 patients IPF = 12 nonIPF ILD = 3 nonILD control = 13 were studied for antiendothelial antibodiesCompared with control subjects serum from IPF patients displayed significantly higher IgG binding to alveolar epithelial cells P = 0041 with a membranous pattern of immunofluorescence However there was no significant difference in immunofluorescence with IgG on endothelial cells P = 0165 In terms of IgM antibodies there was no differential fluorescence observed for either epithelial or endothelial cellsThere is evidence of increased IgG antibodies directed against alveolar epithelium in IPF These antibodies may play a significant role in the pathogenesis of this fibrotic disorder The findings of this study suggest further evaluation of the role of immune mediated alveolar epithelial injury in IPF

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