Seeking and sharing: why the pulmonary fibrosis community engages the web 2.0 environment.

Background: Pulmonary fibrosis (PF) is a rare, progressive disease that affects patients and their loved ones on many levels. We sought to better understand the needs and interests of PF patients and their loved ones (collectively "reader-participants") by systematically analyzing their engagement with the World Wide Web (the current version referred to as Web 2.0).

Identifying the benefits and risks of emerging treatments for idiopathic pulmonary fibrosis: a qualitative study.

Objective: Idiopathic pulmonary fibrosis (IPF) is a rare, progressive, and fatal disease, with very few therapeutic options. Given a paucity of qualitative research to the perspective of patients and other stakeholders in IPF, we sought to identify issues associated with the benefits and risks of emerging treatments and other issues relevant to design of a survey for assessing patient preferences for IPF treatments.

Methods: Semi-structured key informant interviews were conducted, predominately via telephone, with a range of stakeholder perspectives identified through partnership with a national advocacy organization using a combination of purposive and snowball sampling.

Predicting pulmonary fibrosis disease course from past trends in pulmonary function.

Background: The clinical course of idiopathic pulmonary fibrosis (IPF) is characterized by progressive decline in lung function and eventual mortality. We sought to determine if future declines in pulmonary function, mortality, or both can be predicted from prior trends in pulmonary function tests (PFTs).

Methods: Data from 1981 to 2008 on 4,431 PFTs and mortality were analyzed from 734 subjects with IPF.

The peripheral blood transcriptome identifies the presence and extent of disease in idiopathic pulmonary fibrosis.

Rationale: Peripheral blood biomarkers are needed to identify and determine the extent of idiopathic pulmonary fibrosis (IPF). Current physiologic and radiographic prognostic indicators diagnose IPF too late in the course of disease. We hypothesize that peripheral blood biomarkers will identify disease in its early stages, and facilitate monitoring for disease progression.

A common MUC5B promoter polymorphism and pulmonary fibrosis.

Background: The mutations that have been implicated in pulmonary fibrosis account for only a small proportion of the population risk.

Methods: Using a genomewide linkage scan, we detected linkage between idiopathic interstitial pneumonia and a 3.4-Mb region of chromosome 11p15 in 82 families.

Heart rate recovery after 6-min walk test predicts survival in patients with idiopathic pulmonary fibrosis.

Background: In patients with idiopathic pulmonary fibrosis (IPF), our objectives were to identify predictors of abnormal heart rate recovery (HRR) at 1 min after completion of a 6-min walk test (6MWT) [HRR1] and 2 min after completion of a 6MWT (HRR2), and to determine whether abnormal HRR predicts mortality.

Methods: From 2003 to 2008, we identified IPF patients who had been evaluated at our center (n = 76) with a pulmonary physiologic examination and the 6MWT. We used logistic regression to identify predictors of abnormal HRR, the product-limit method to compare survival in the sample stratified on HRR, and Cox proportional hazards analysis to estimate the prognostic capability of abnormal HRR.

Molecular phenotypes distinguish patients with relatively stable from progressive idiopathic pulmonary fibrosis (IPF).

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive, chronic interstitial lung disease that is unresponsive to current therapy and often leads to death. However, the rate of disease progression differs among patients. We hypothesized that comparing the gene expression profiles between patients with stable disease and those in which the disease progressed rapidly will lead to biomarker discovery and contribute to the understanding of disease pathogenesis.