Abbreviation: PF = pulmonary fibrosis The intention 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 common affected member.

Abbreviation: PF = pulmonary fibrosis

The intention 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 common affected member. Families with at least pair members affected by PF were recruited in consequence of a network of collaborators and advertisement onward the Internet (www.fpf.duke.edu). Data forward 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 covered with a questionnaire, diffusion capacity of the lung for carbon monoxide, and chest radiography. Thirty-eight families affected through PF were identified between 1998 and 2001 and are generally 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). prospect to known fibrotic agents, as it is as asbestos, silica, or forest dust, was recorded in 14 patients (22% of cases with available data). The mean age at diagnosis is 58 years (SD = 157 years), and the mean age at death is 61 years (SD = 157 years). The diagnosis of PF was confirmed in 59 patients (47%) at either an open-lung biopsy/autopsy or by the agency of meeting American Thoracic Society criteria for the diagnosis of PF In the 18 families with at least couple documented cases, PF occurred in three or more family members in 9 families. Fourteen families have at least single affected sibling pair; 4 families have affected parent-child pairs, including individual 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.

* From Duke University Medical Center Durham, NC; and National Jewish Medical and Research Center Denver CO storeed by National Heart, Lung, and posterity Institute grant HL-67467.

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

...