The impact of antifibrotic use on long-term clinical outcomes in the pulmonary fibrosis foundation registry.

Background: Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease (ILD) with a high mortality rate. The antifibrotic medications pirfenidone and nintedanib have been in use since 2014 for this disorder and are associated with improved rate of lung function decline. Less is known about their long-term outcomes outside of the clinical trial context.

Real-World Clinical Outcomes Based on Body Mass Index and Annualized Weight Change in Patients with Idiopathic Pulmonary Fibrosis.

Introduction: Identification of clinical characteristics associated with prognosis for idiopathic pulmonary fibrosis (IPF) may help to guide management decisions. This analysis utilized data from the Pulmonary Fibrosis Foundation Patient Registry to examine the relationships between clinical outcomes and both body mass index (BMI) at study enrollment (hereafter referred to as baseline BMI) and annualized percent change in body weight in patients with IPF in a real-world setting.

Methods: The following outcomes over 24 months were stratified by baseline BMI and annualized percent change in body weight: all-cause mortality; annualized change in percent predicted forced vital capacity (%FVC), percent predicted diffusing capacity for carbon monoxide, and 6-min walk distance; all-cause and respiratory-related hospitalizations; and acute exacerbations.

Using forced vital capacity (FVC) in the clinic to monitor patients with idiopathic pulmonary fibrosis (IPF): pros and cons.

Introduction: Forced vital capacity (FVC) decline is predictive of mortality in patients with idiopathic pulmonary fibrosis (IPF) and has been used as a clinical trial endpoint to define disease progression. How to interpret FVC findings in an individual patient with IPF in the real-world setting amid uncertainty about the measurement accuracy and variability has not been well established.

Areas Covered: This review highlights the challenges and limitations of using FVC in the clinic to monitor disease progression in patients with IPF.

The Pulmonary Fibrosis Foundation Patient Registry. Rationale, Design, and Methods.

Detailed understanding of longitudinal behavior, response to therapy, and applicable biomarkers for interstitial lung diseases (ILDs) is lacking. There is a need for a large multicenter registry that provides researchers and clinicians access to well-characterized data not limited to patients with idiopathic pulmonary fibrosis. The Pulmonary Fibrosis Foundation Patient Registry (PFF-PR) is a database that collects baseline and longitudinal demographic and clinical information about patients with ILDs in the United States.

Effect of pirfenidone on gastric emptying in a rat model.

Introduction: Gastrointestinal (GI) adverse events (AEs) are commonly reported in patients with idiopathic pulmonary fibrosis who are treated with pirfenidone. Taking pirfenidone with a substantial amount of food or dividing the dose over the course of a meal has been reported to reduce the frequency of GI AEs in clinical practice. In humans, the maximum plasma concentration (C) of pirfenidone was reduced when the drug was taken with food compared with the fasting state, and the lower C was associated with a reduction in GI AE rates.

An interprofessional approach to reducing the overutilization of stress ulcer prophylaxis in adult medical and surgical intensive care units.

Background: Overutilization of stress ulcer prophylaxis (SUP) in the intensive care unit (ICU) is common. Acid-suppressive therapies routinely used for SUP are best reserved for patients with greatest risk of clinically important bleeding as they have been associated with nosocomial pneumonia, Clostridium difficile infection and increased hospital cost.

Objective: The primary objective was to reduce inappropriate utilization of SUP in 2 adult medical and surgical ICU settings at the University of California, San Francisco Medical Center.