Fast Five Quiz: Interstitial Lung Disease Progressive Fibrosing Management

Zab Mosenifar, MD


March 06, 2023

The most common, and for most patients with ILD the most debilitating, symptom of ILD is dyspnea. The American Thoracic Society (ATS) clinical practice guidelines recommend long-term oxygen for patients with ILD who have severe chronic resting hypoxemia, or PaO2 ≤ 55 mmHg. Studies have shown that the use of home oxygen may increase exercise tolerance, decrease dyspnea, and improve health-related quality of life in patients with ILDs.

The main elements of supportive care for patients with ILD include pulmonary rehabilitation. Pulmonary rehabilitation may allow for reversal of muscle deconditioning, ultimately enabling patients to walk farther and with less dyspnea. Although pulmonary rehabilitation may improve functional status, it has not been shown to not slow disease progression or improve survival in patients with ILD.

Current guidelines recommend against the use of antacid therapy for the purpose of improving respiratory symptoms in patients with PPF. PPIs have not been shown to have a significant effect on disease progression or to reduce all-cause mortality, respiratory-related mortality, or respiratory-related hospitalization.

Pulmonary function testing may be performed every 3-6 months in the course of long-term monitoring.

Learn more about the treatment of PF-ILD.


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