Published ahead of print on July 19, 2007, doi:10.1164/rccm.200702-254OC Am. J. Respir. Crit. Care Med., Volume 176, Number 7, October 2007, 698-705 A more recent version of this article appeared on October 1, 2007
Submitted on February 14, 2007 Early Interstitial Lung Disease in Familial Pulmonary FibrosisIvan O Rosas1,1 National Institutes of Health, Pulmonary-Critical Care Medicine Branch, Bethesda, MD, USA, 2 Diagnostic Radiology Department, National Institutes of Health, Clinical Center, Bethesda, MD, USA, 3 Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC, USA, 4 National Institutes of Health, National Heart, Lung, and Blood Institute, Flow Cytometry Core Facility, Bethesda, MD, USA, 5 National Institutes of Health, National Cancer Institute, Surgery Branch, Bethesda, MD, USA, 6 National Institutes of Health, National Human Genome Research Institute, Medical Genetics Branch, Bethesda, MD, USA * To whom correspondence should be addressed. E-mail: gochuicb{at}mail.nih.gov.
Rationale: Identification of early, asymptomatic interstitial lung disease (ILD) in populations at risk of developing IPF may improve the understanding of the natural history of idiopathic pulmonary fibrosis (IPF). Objectives: To determine clinical, radiographic, physiologic and pathologic features of asymptomatic ILD in family members of patients with familial IPF. Methods: 164 subjects from 18 kindreds affected with familial IPF were evaluated for ILD. Bronchoalveolar lavage (BAL) cells were analyzed using flow cytometry. Lung biopsies were performed in six subjects with asymptomatic ILD. Measurements and Main Results: High-resolution computed tomography (HRCT) abnormalities suggesting ILD were identified in 31 (22%) of 143 asymptomatic subjects. Subjects with asymptomatic ILD were significantly younger than subjects with known familial IPF (p<0.001) and significantly older than related subjects without lung disease (p<0.001). A history of smoking was identified in 45% of subjects with asymptomatic ILD and in 67% of subjects with familial IPF; these percentages were significantly higher than that of related subjects without lung disease (23%) (p=0.02 and p<0.001, respectively). Percentages of activated CD4+ lymphocytes were significantly higher in BAL cells from subjects with asymptomatic ILD compared to related subjects without lung disease (p<0.001). Lung biopsies performed in subjects with asymptomatic ILD revealed diverse histologic subtypes. Conclusions: Asymptomatic ILD in individuals at risk of developing familial IPF can be identified using HRCT scan of the chest, especially in those with a history of smoking. Lung biopsies from individuals in this cohort with early asymptomatic lung disease demonstrate various histologic subtypes of ILD. Key words: IPF, ILD, HRCT, bronchoalveolar lavage, asymptomatic ILD
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