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Idiopathic Pulmonary Fibrosis Can Be an Autosomal Dominant Trait in Some Families*

Annis Marney, BA; Kirk B. Lane, PhD; John A. Phillips, III, MD; David J. Riley, MD, FCCP; James E. Loyd, MD
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*From the Vanderbilt University Medical School (Ms. Marney, and Drs. Lane, Phillips, and Loyd), Nashville, TN; and Robert Wood Johnson Medical School (Dr. Riley), Piscataway, NJ.

Correspondence to: Annis Marney, BA, Division of Pulmonary and Critical Care Medicine, Vanderbilt University Center, Nashville, TN 37232-2605



Chest. 2001;120(1_suppl):S56. doi:10.1378/chest.120.1_suppl.S56
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Investigation of diseases such as idiopathic pulmonary fibrosis (IPF) occurring in large families offers the possibility to uncover a central pathogenetic basis of disease. One of the first reported families with IPF has been described by many different authors.13 Since that time, 43 families have appeared in the literature. In 1965, the original, large family had suffered eight proven cases of IPF, including seven female patients and one male patient; their ages at death ranged from 38 to 52 years. Twin sisters died of IPF at ages 46 and 47 years, respectively, after living in different states for 25 years, suggesting that environmental exposure is relatively unimportant. A psychiatrist father died at the age of 54 years and his son died at the age of 32 years of IPF. Three other family members (one female and two male subjects; age range at death, 32 to 73 years) died from respiratory disease compatible with but not proven to be IPF.

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