IPF Common Interstitial Lung Disease in CARE-PF Registry Enrolling in Canada

The Canadian Registry for Pulmonary Fibrosis (CARE-PF) enrolled in its first 18 months more than 1,200 people with interstitial lung disease (ILD), with more than half having either idiopathic pulmonary fibrosis (IPF) or connective tissue disease-associated interstitial lung disease (CTD-ILD).

Data collected through the CARE-PF registry could be useful in better understanding pulmonary fibrosis, and improving the care of people with this disease.

In a recent article published in the journal BMC Pulmonary Medicine, researchers described the baseline features of people taking part in CARE-PF. Their article is titled “Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis.”

Enrollment in CARE-PF, which started in January 2016, is open to anyone 18 or older with an ILD, who is able to complete questionnaires in English or French, and capable of giving informed consent. The registry’s goal is to provide a broad overview of people with ILD in Canada, and to observe changes over time (a natural history study). Recruitment is being done at six specialized ILD centers across the country.

As of July 2017 (the cut-off point for this study’s analysis), 1,285 people with ILDs had enrolled — representing the largest research cohort of people with ILD in Canada, and one of the largest in the world, the researchers wrote.

Their overview detailed participants’ relevant characteristics and comorbidities.

Specific ILD diagnoses include CTD-ILD (33.3%), IPF (24.7%), unclassifiable ILD (22.3%), chronic hypersensitivity pneumonitis (7.5%), sarcoidosis (3.2%), non-IPF idiopathic interstitial pneumonia (3.0%), and other ILDs (6.0%).

The group generally consists of older adults (mean age of 64.8), with a roughly similar proportion of men and women. Most of the participants enrolled (62.9%) are or were smokers.

A few general differences among the diagnoses were noted. “Compared to other diagnoses, patients with IPF and unclassifiable ILD were older, patients with IPF were more frequently male, and patients with sarcoidosis had higher lung function,” the researchers noted.

Differences were also seen among the diagnoses in terms of rate of oxygen treatment use, from 5.3% in sarcoidosis to 34.1% in IPF.

The number of comorbidities (according to the Charlson Comorbidity Index, or CCI) were similar across the ILD groups. In total, 81.1% of these people had a mean CCI score of 2 or less, meaning no or only a few other diseases or conditions. But the researchers questioned the index’s applicability in this group.

The most common patient-reported comorbidities were gastroesophageal reflux disease (GERD; 26.6%), chronic obstructive pulmonary disease (COPD; 23.1%), diabetes (15.2%), and obstructive sleep apnea (14.5%).

The registry is also collecting data on exposure to environmental hazards that might affect lung health, from down feathers to mold, hot tubs, and asbestos.

The team found that patient-reported exposures were common in all ILD diagnoses, “with organic exposures more frequently endorsed than inorganic ones,” they wrote. Organic exposures included down feathers, mold, and water.

CARE-PF represents a wide sampling of ILD patients in Canada. The team found that “CTD-ILD, IPF, and unclassifiable ILD made up 80% of ILD diagnoses,” and noted that “comorbidities were frequent,” and the CCI score has limited ability “to represent the extent and spectrum of comorbid diseases in this population.”

The researchers believe that following these patients over the long term will offer further insights into ILDs and clinical outcomes.