AnneMarie Mc Laughlin, Rosemary O’Brien, Seamas C. Donnelly
{"title":"Familial idiopathic pulmonary fibrosis occurring in four members of a family","authors":"AnneMarie Mc Laughlin, Rosemary O’Brien, Seamas C. Donnelly","doi":"10.1016/j.rmedx.2007.01.003","DOIUrl":null,"url":null,"abstract":"<div><p>Idiopathic pulmonary fibrosis<span> (IPF) is a progressive fatal lung disease characterised by persistent pulmonary inflammation. Approximately 2% of cases are familial. Herein, we describe a family in which four members over two generations developed IPF. Of particular interest, the age of onset was lower in the second generation affected by the disease. Familial IPF offers a unique opportunity to study the proinflammatory processes implicated in IPF.</span></p></div>","PeriodicalId":101082,"journal":{"name":"Respiratory Medicine Extra","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2007-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rmedx.2007.01.003","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Respiratory Medicine Extra","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1744904907000021","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive fatal lung disease characterised by persistent pulmonary inflammation. Approximately 2% of cases are familial. Herein, we describe a family in which four members over two generations developed IPF. Of particular interest, the age of onset was lower in the second generation affected by the disease. Familial IPF offers a unique opportunity to study the proinflammatory processes implicated in IPF.
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