家族性特发性肺纤维化发生在一个家庭的四名成员中

Respiratory Medicine Extra Pub Date : 2007-01-01 DOI:10.1016/j.rmedx.2007.01.003

AnneMarie Mc Laughlin, Rosemary O’Brien, Seamas C. Donnelly

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摘要

特发性肺纤维化(IPF)是一种以持续肺部炎症为特征的进行性致死性肺病。大约2%的病例是家族性的。在这里，我们描述了一个家庭，其中四名成员超过两代发展IPF。特别有趣的是，患病第二代的发病年龄较低。家族性IPF为研究IPF中涉及的促炎过程提供了一个独特的机会。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Familial idiopathic pulmonary fibrosis occurring in four members of a family

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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Respiratory Medicine Extra

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