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

Abstract

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. The objective of this study is to describe the patient population, data collection process, and opportunities for retrospective and prospective research with the PFF-PR. Individuals 18 years or older who had ILD diagnosed and who were seen at PFF-PR centers who provided informed consent were eligible to participate. Baseline and longitudinal demographic, spirometric, radiographic, morbidity, and mortality data are recorded into a secure electronic data capture system. Starting in 2016, the PFF-PR has collected data on 2,003 patients at 42 clinical sites in the United States. At the time of enrollment, the mean age of participants was 68 years old. Most (62%) of participants were male, and 58% had a positive smoking history. The mean forced vital capacity was 69% predicted, and the mean diffusing capacity of the lung for carbon monoxide was 43% predicted. Forty-one percent of patients were using supplemental oxygen, and 39% were on antifibrotic therapy. Reasons for attrition were mostly death or transplant, with low rates of loss to follow-up or withdrawal. The PFF-PR is a large multicenter United States-based registry that provides researchers and clinicians access to well-characterized ILD patient data.

Keywords: idiopathic pulmonary fibrosis; interstitial; lung diseases; patient registries; registries.

Figure 1.

Geographical representation of Pulmonary Fibrosis Foundation Care Center sites.

Figure 2.

Interstitial lung disease subgroups at time of enrollment. CVD-ILD = collagen vascular disease-associated interstitial lung disease; IIP = idiopathic interstitial pneumonia; IPF = idiopathic pulmonary fibrosis.

Figure 3.

Baseline mean pulmonary function testing (PFT) data at the time of enrollment. CVD-ILD = collagen vascular disease-associated interstitial lung disease; Dl _CO = diffusing capacity of the lungs for carbon monoxide; FEV₁ = forced expiratory volume in 1 second; FVC = forced vital capacity; HP = hypersensitivity pneumonitis; IIP = idiopathic interstitial pneumonia; IPF = idiopathic pulmonary fibrosis.

Figure 4.

Frequency of medical comorbidities at the time of enrollment. CVD-ILD = collagen vascular disease-associated interstitial lung disease; GERD = gastroesophageal reflux disease; HP = hypersensitivity pneumonitis; IIP = idiopathic interstitial pneumonia; IPF = idiopathic pulmonary fibrosis.

Figure 5.

Diagnostic methods/processes associated with each interstitial lung disease subgroup. More than one diagnostic method/process could have been used per patient to reach a diagnosis. CVD-ILD = collagen vascular disease-associated interstitial lung disease; HP = hypersensitivity pneumonitis; IIP = idiopathic interstitial pneumonia; ILD = interstitial lung disease; IPF = idiopathic pulmonary fibrosis.

Figure 6.

Medical therapies associated with each interstitial lung disease subgroup. CVD-ILD = collagen vascular disease-associated interstitial lung disease; HP = hypersensitivity pneumonitis; IIP = idiopathic interstitial pneumonia; IPF = idiopathic pulmonary fibrosis.

Figure 7.

Clinical trial participation in the past 12 months by interstitial lung disease subgroup. CVD-ILD = collagen vascular disease-associated interstitial lung disease; HP = hypersensitivity pneumonitis; IIP = idiopathic interstitial pneumonia; IPF = idiopathic pulmonary fibrosis.