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Published ahead of print on August 18, 2005, doi:10.1164/rccm.200408-1104OC

Am. J. Respir. Crit. Care Med., Volume 172, Number 9, November 2005, 1146-1152

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Submitted on August 23, 2004
Accepted on August 16, 2005

The Clinical and Pathologic Features of Familial Interstitial Pneumonia (FIP)

Mark P Steele1*, Marcy C Speer2, James E Loyd3, Kevin K Brown4, Aretha Herron1, Susan H Slifer2, Lauranell H Burch1, Momen M Wahidi1, John A Phillips III3, Thomas A Sporn5, H. Page McAdams6, Marvin I Schwarz4, and David A Schwartz7

1 Department of Medicine and Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, NC, USA, 2 Center for Human Genetics, Duke University Medical Center, Durham, NC, USA, 3 Vanderbilt University School of Medicine, Nashville, TN, USA, 4 National Jewish Medical and Research Center, Denver, CO, USA; University of ColoradoHealth Sciences Center, Denver, CO, USA, 5 Department of Pathology, Duke University Medical Center, Durham, NC, USA, 6 Department of Radiology, Duke University Medical Center, Durham, NC, USA, 7 Department of Medicine and Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, NC, USA; VA Medical Centers, Durham, NC, USA

* To whom correspondence should be addressed. E-mail: mark.steele{at}duke.edu.

Rationale: Several lines of evidence suggest that genetic factors and environmental exposures play a role in the development of pulmonary fibrosis. Objectives: We evaluated families with 2 or more cases of idiopathic interstitial pneumonia (IIP) in first degree family members (familial interstitial pneumonia or FIP), and identified 111 families with FIP having 309 affected and 360 unaffected individuals. Methods: The presence of probable or definite FIP was based on medical record review in 28 (9.2%); clinical history, DLCO, and CXR in 16 (5.2%); clinical history, DLCO, and HRCT scan in 191 (61.8%); clinical history and surgical lung biopsy (SLB) in 56 (18.1%); and clinical history and autopsy in 18 (5.8%). Results: Older age (68.3 vs. 53.1; P<0.0001), male gender (55.7% vs. 37.2%; P<0.0001), and having ever smoked cigarettes (67.3% vs. 34.1%; P<0.0001) were associated with the development of FIP. After controlling for age and gender, having ever smoked cigarettes remained strongly associated with the development of FIP (odds ratioadj=3.6; 95% CI=1.3-9.8). Evidence for aggregation of disease was highly significant (P<0.001) among sibling pairs, and 20 pedigrees demonstrated vertical transmission, consistent with autosomal dominant inheritance. 45% of pedigrees demonstrated phenotypic heterogeneity, with some pedigrees demonstrating several subtypes of IIP occurring within the same families. Conclusions: These findings suggest that FIP may be caused by an interaction between a specific environmental exposure and a gene (or genes) that predisposes to the development of several subtypes of IIP.
Key words: pulmonary fibrosis, familial pulmonary fibrosis, genetics, cigarette smoking




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