Pirfenidone in fibrotic hypersensitivity pneumonitis: a double-blind, randomised clinical trial of efficacy and safety.

Background: Fibrotic hypersensitivity pneumonitis (FHP) is an irreversible lung disease with high morbidity and mortality. We sought to evaluate the safety and effect of pirfenidone on disease progression in such patients.

Methods: We conducted a single-centre, randomised, double-blinded, placebo-controlled trial in adults with FHP and disease progression.

Design and rationale of a randomised, double-blind trial of the efficacy and safety of pirfenidone in patients with fibrotic hypersensitivity pneumonitis.

Hypersensitivity pneumonitis (HP) is an immunologically mediated form of lung disease resulting from inhalational exposure to any of a large variety of antigens. A subgroup of patients with HP develops pulmonary fibrosis (fibrotic HP; FHP), a significant cause of morbidity and mortality. This study will evaluate the safety and efficacy of the antifibrotic pirfenidone in treating FHP.

Tracking dyspnea up to supplemental oxygen prescription among patients with pulmonary fibrosis.

Background: Dyspnea is the hallmark symptom of pulmonary fibrosis. Supplemental oxygen (O) is prescribed to many patients with pulmonary fibrosis in hopes of alleviating dyspnea and improving physical functioning. We used response data from the University of California San Diego Shortness of Breath Questionnaire (UCSD) which was administered monthly in the context of a longitudinal, observational study to plot a rich trajectory for dyspnea over time in patients with pulmonary fibrosis.

Looking ahead and behind at supplemental oxygen: A qualitative study of patients with pulmonary fibrosis.

Rationale: Supplemental oxygen is prescribed to patients with pulmonary fibrosis to normalize oxygen saturations, decrease symptoms and improve quality of life. Along with potential benefits, patients face challenges as they incorporate oxygen into their lives.

Objective: Our aim was to better understand the perceptions and experiences of patients with pulmonary fibrosis as they confronted the possibility and realities of using supplemental oxygen.

Informal caregivers experience of supplemental oxygen in pulmonary fibrosis.

Background: Patients prescribed supplemental oxygen (O) therapy face challenges as they adjust to being constantly "tethered" to an oxygen delivery device. Informal caregivers (ICs) of patients with pulmonary fibrosis (PF) face their own, often overlooked hardships when O is brought into their home and added to their lives. Our aim was to understand the multiple effects of supplemental oxygen therapy on ICs of patients with PF.

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).

Development of ATAQ-LAM: a tool to assess quality of life in Lymphangioleiomyomatosis.

Background: Lymphangioleiomyomatosis (LAM) is a progressive lung disease that impairs health-related quality of life (HRQL).

Objective: To develop and conduct initial testing of ATAQ-LAM (A Tool to Assess Quality of Life in LAM).

Methods: A pilot version of the questionnaire was administered to respondents with LAM.

Protocol for a mixed-methods study of supplemental oxygen in pulmonary fibrosis.

Background: Little is known about whether or how supplemental oxygen affects patients with pulmonary fibrosis.

Methods/design: A mixed-methods study is described. Patients with pulmonary fibrosis, informal caregivers of pulmonary fibrosis patients and practitioners who prescribe supplemental oxygen will be interviewed to gather data on perceptions of how supplemental oxygen impacts patients.

"Getting stuck with LAM": patients perspectives on living with lymphangioleiomyomatosis.

Background: Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that affects almost exclusively women and is most often diagnosed before menopause. The main symptom of LAM is shortness of breath. LAM patients' perceptions of how the disease impacts their lives is largely unknown, but such information could be useful to generate patient reported outcome measures for use in drug trials (or other research studies) and to formulate interventions aimed at easing the burdens LAM imposes on patients.