title: Early interstitial lung disease in familial pulmonary fibrosis.
abstract: BACKGROUND
  Identification of early, asymptomatic interstitial lung disease (ILD) in populations at risk of developing idiopathic pulmonary fibrosis (IPF) may improve the understanding of the natural history of IPF.
  
  
  OBJECTIVE
  To determine clinical, radiographic, physiologic, and pathologic features of asymptomatic ILD in family members of patients with familial IPF.
  
  
  METHODS
  One hundred sixty-four subjects from 18 kindreds affected with familial IPF were evaluated for ILD. Bronchoalveolar lavage fluid cells were analyzed using flow cytometry. Lung biopsies were performed in six subjects with asymptomatic ILD.
  
  
  RESULTS
  High-resolution computed tomography 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 bronchoalveolar lavage fluid cells from subjects with asymptomatic ILD compared with 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 high-resolution computed tomography 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.
authors.affiliation: Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892-1590, USA.
authors.forename: Ivan O
authors.initials: IO
authors.lastname: Rosas
authors.forename: Ping
authors.initials: P
authors.lastname: Ren
authors.forename: Nilo A
authors.initials: NA
authors.lastname: Avila
authors.forename: Catherine K
authors.initials: CK
authors.lastname: Chow
authors.forename: Teri J
authors.initials: TJ
authors.lastname: Franks
authors.forename: William D
authors.initials: WD
authors.lastname: Travis
authors.forename: J Philip
authors.initials: JP
authors.lastname: McCoy
authors.forename: Rose M
authors.initials: RM
authors.lastname: May
authors.forename: Hai-Ping
authors.initials: HP
authors.lastname: Wu
authors.forename: Dao M
authors.initials: DM
authors.lastname: Nguyen
authors.forename: Mauricio
authors.initials: M
authors.lastname: Arcos-Burgos
authors.forename: Sandra D
authors.initials: SD
authors.lastname: MacDonald
authors.forename: Bernadette R
authors.initials: BR
authors.lastname: Gochuico
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000150:complications / Q000235:genetics*; D012907:Smoking