During the session Managing the IPF Patient Across the Disease Spectrum: An Evidence-Based Update, a pair of pulmonary specialists outlined the challenges in treating idiopathic pulmonary fibrosis (IPF), including key management decisions at the time of diagnosis and approaches to managing acute exacerbation.

The session is available for on-demand viewing to registered CHEST 2021 attendees through October 1, 2022.

Amy Hajari Case, MD, FCCP, Director of the Interstitial Lung Disease Program at Piedmont Healthcare in Atlanta, opened the session with a discussion on how to manage a patient with a new IPF diagnosis.

“When we think about the patient with (IPF), our minds immediately go to antifibrotic therapy,” she said. “But what I want us to think about is comprehensive management of the IPF patient, meaning that the antifibrotic therapies that we have are only part of the management plan for our patients.”

A comprehensive management plan might include treatment of comorbidities, smoking cessation, preventive care, education and support groups, pulmonary rehabilitation, research opportunities, transplant evaluation, and supportive care, Dr. Case noted. She also advocates for a shared decision-making approach to establishing each patient’s plan.

If antifibrotic therapy is used, the choice between the two antifibrotic drugs with US Food and Drug Administration approval for the treatment of IPF—pirfenidone and nintedanib—is not clear cut. Both options slow disease progression as measured by forced vital capacity, but neither is expected to change any symptoms of IPF, Dr. Case explained. When selecting which drug to use, she said adverse events reported with each option should be considered. Of patients taking nintedanib in a phase 3 trial, 62% reported experiencing diarrhea, 24% nausea, and 15% abdominal pain. Among patients taking pirfenidone in a separate phase 3 trial, 36% reported nausea, 30% a rash, and 27% an upper respiratory tract infection.

At regular intervals following a diagnosis of IPF, the patient should undergo pulmonary function testing, a 6-minute walk test to assess functional status and the need for supplemental oxygen, liver function testing, and follow-up imaging, Dr. Case said.

Tejaswini Kulkarni, MD, MBBS, Assistant Professor and Director of the Interstitial Lung Disease Program at the University of Alabama at Birmingham, outlined current and emerging therapies for managing the acute exacerbation of IPF (AE-IPF), including antimicrobial therapy, bronchoscopy, oxygen therapy, mechanical ventilation, and antifibrotic drugs.

“As the most common cause of death in most of the IPF cohorts, the implications of AE are profound, but unfortunately there are no proven or effective treatments,” Dr. Kulkarni said. “The severity of AEs is variable, and these do impact treatment decisions to some extent, but the currently used therapeutic approaches are all based on expert consensus or small uncontrolled or retrospective studies.”

Corticosteroids are the most common treatment for patients with AE-IPF.

“The idea behind that is that the pathological finding in an acute exacerbation of IPF is diffuse alveolar damage, and the utilization of corticosteroids is based on the extrapolation of its effects that have been seen on pulmonary inflammation in the acute lung injury models and the ARDSM models,” Dr. Kulkarni said, adding that there are no specific recommendations regarding dose, route of administration, or duration of corticosteroid use in AE-IPF.

She cited a prospective study that reported avoiding steroids may reduce adverse events associated with immunosuppression for patients with AE-IPF. A single-center retrospective study of 82 patients showed no significant difference in in-hospital mortality among those who were given steroids and those who were not. However, the all-cause mortality of the cohort receiving steroids was increased shortly after hospital discharge, Dr. Kulkarni said.

The Autoantibody Reduction for Acute Exacerbations of Idiopathic Pulmonary Fibrosis (STRIVE-IPF) study, which is currently enrolling phase 2 participants, is studying an autoantibody reduction protocol for AE-IPF.

“AE-IPF confers a poor prognosis. The revised diagnostic criteria have shifted to more of a pathophysiology basis rather than an etiology basis, but the management of AE-IPF really remains enigmatic, so ultimately, focus on the best supportive care for your patient,” Dr. Kulkarni said.