Clinical and pathologic features of familial interstitial pneumonia.

ABSTRACT:

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 among 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 cases (9.1%); clinical history, diffusing capacity of carbon monoxide (DL(CO)), and chest X-ray in 16 cases (5.2%); clinical history, DL(CO), and high-resolution computed tomography chest scan in 191 cases (61.8%); clinical history and surgical lung biopsy in 56 cases (18.1%); and cl

SUBMITTER: Steele MP 

PROVIDER: S-EPMC2718398 | biostudies-literature | 2005 Nov

REPOSITORIES: biostudies-literature