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Familial Pulmonary Fibrosis in the United States*

Momen M. Wahidi, MD; Marcy C. Speer, PhD; Mark P. Steele, MD, FCCP; Kevin K. Brown, MD, FCCP; Marvin I. Schwarz, MD, FCCP; David A. Schwartz, MD, MPH
Author and Funding Information

*From Duke University Medical Center, Durham, NC; and National Jewish Medical and Research Center, Denver, CO.

Correspondence to: Momen M. Wahidi, MD, Duke University Medical Center, DUMC 2629, Room 275 MSRB, Research Dr, Durham NC 27710; e-mail: wahid001@mc.duke.edu



Chest. 2002;121(3_suppl):30S. doi:10.1378/chest.121.3_suppl.30S
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The purpose of this study is to describe the epidemiologic and clinical characteristics and the familial aggregation of pulmonary fibrosis (PF) in a series of families with more than one affected member. Families with at least two members affected by PF were recruited through a network of collaborators and advertisement on the Internet (www.fpf.duke.edu). Data on affected members were collected, including a standardized clinical history and risk factor questionnaire, pedigree, diffusion capacity of the lung for carbon monoxide, chest radiography, high-resolution chest CT, and lung biopsy (when available). Asymptomatic relatives were screened with a questionnaire, diffusion capacity of the lung for carbon monoxide, and chest radiography. Thirty-eight families affected by PF were identified between 1998 and 2001 and are currently under investigation. The total number of affected individuals is 125 (males, 56%; females, 44%). The predominant race is white (Caucasian, 92%; Hispanic, 6%). Smoking was identified in 33 patients (51% of cases with available data). Exposure to known fibrotic agents, such as asbestos, silica, or wood dust, was recorded in 14 patients (22% of cases with available data). The mean age at diagnosis is 58 years (SD = 15.7 years), and the mean age at death is 61 years (SD = 15.7 years). The diagnosis of PF was confirmed in 59 patients (47%) by either an open-lung biopsy/autopsy or by meeting American Thoracic Society criteria for the diagnosis of PF. In the 18 families with at least two documented cases, PF occurred in three or more family members in 9 families. Fourteen families have at least one affected sibling pair; 4 families have affected parent-child pairs, including one pair with an affected father and son; and five pedigrees include more distantly related affected relative pairs. The familial aggregation documented in these families is consistent with a genetic basis in at least a subset of PF cases.

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