Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern.

ABSTRACT:

Background

Idiopathic pulmonary fibrosis (IPF) is characterized by the insidious onset of dyspnea or cough. However, a subset of patients has a short duration of symptoms with rapid progression to end-stage disease. In this study, we evaluated clinical and molecular features of "rapid" and "slow" progressors with IPF.

Methods and findings

26 patients with <6 months of symptoms before first presentation [rapid progressors] and 88 patients with >24 months of symptoms [slow progressors] were studied. Survival was analyzed by the Kaplan-Meyer method and proportional hazard's model. Lung microarrays and tissue proteins were measured in a subset of patients. No differences were found in age, physiologic impairment and bronchoalveolar lavage (BAL) cellular profile. There were more

SUBMITTER: Selman M 

PROVIDER: S-EPMC1868965 | biostudies-literature | 2007 May

REPOSITORIES: biostudies-literature