Implementation of Guidelines for Idiopathic Pulmonary Fibrosis Management

Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
How effective are international guidelines for the management of idiopathic pulmonary fibrosis?

Limited data are available regarding the degree to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). In the current study published in Respiratory Medicine, researchers analyzed the extent to which IPF management guidelines have been used in the US Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry, as well as links between utilization and clinical outcomes.

They found that management guidelines were more frequently implemented in IPF patients with greater disease severity. No relationship between implementation of management guidelines and clinical outcomes was observed, after compensating for disease severity. In the aggregate, the investigators discovered generally low implementation of management guidelines in their analysis.

The investigators analyzed the implementation of 8 recommendations in clinical practice guidelines within 6 months of registry enrollment. These included the following: 1) visit to a specialized clinic; 2) pulmonary function testing; 3) use of oxygen in patients with resting hypoxemia; 4) use of oxygen in patients with exercise-induced hypoxemia; 5) referral for pulmonary rehabilitation; 6) treatment of gastro-esophageal reflux disease; 7) initiation of anti-fibrotic therapy; 8) and referral for lung transplant evaluation. They then calculated an implementation score based on the number of recommendations attained divided by the number for which the patient was eligible.

In 727 patients, the median implementation score was 0.6. Patients who scored more than 0.6 had greater disease severity compared with those with lower scores. The lowest scores were for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). Higher unadjusted scores were correlated with a greater risk of death, death or lung transplant, and hospitalization. No significant associations, however, were demonstrated in adjusted models.

The Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry is a multi-center registry of US patients diagnosed with IPF. The goal of this registry is to elucidate the course of IPF, how it impacts patients, and status quo practices in diagnosis and patient care. 

In other findings, only 6.3% of patients had all eligible recommendations implemented. The recommendation for oxygen therapy in those with resting hypoxemia was implemented in three-quarters of the participants.

The current study has various limitations. First, the sites in the IPF-PRO Registry represent centers with experience in the management of IPF and probably don’t reflect general US pulmonary practices. Second, this analyses was derived from referrals and prescriptions based on data extracted from medical records; it was not based on the actual use of therapies. Third, a source of bias could be that patients with more severe IPF were eligible for more interventions, which could impact implementation. Fourth, the researchers could not assess the reasons why certain recommendations were or were not implemented for a given patient.

“Guidelines for the management of IPF need to be complemented with clinical expertise and an understanding of patients’ values and preferences in order to provide the best care for an individual patient,” said the authors. “Use of simple checklists for implementation of management guidelines does not account for the nuanced and individualized care decisions made for patients with IPF in clinical practice and does not equate to quality of care.”

The authors stress that failure to implement a guideline may not be a “failure” of the site to comply with best practices. Legitimate reasons for not implementing a guideline could exist such as patient choice.

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures


De Andrade JA, Kulkami T, Neely ML, et al. Implementation of guideline recommendations and outcomes in patients with idiopathic pulmonary fibrosis: data from the IPF-PRO Registry. Respir Med. Published online September 29, 2021. doi:10.1016/j.rmed.2021.106637