PG 2025: Pulmonary Literature Review and Interactive Complex Case Presentations (PG)-Chan KM - Pulmonary Literature Review and Interactive Complex Case Presentations

Chan KM - Pulmonary Literature Review and Interactive Complex Case Presentations

Pdf Summary

The 2025 Interstitial Lung Disease (ILD) Year in Review, presented by Dr. Kevin M. Chan, summarizes key advances in ILD, focusing on idiopathic pulmonary fibrosis (IPF), progressive pulmonary fibrosis (PPF), pulmonary sarcoidosis, and interstitial lung abnormalities (ILA).<br /><br />Nerandomilast, an oral phosphodiesterase 4B inhibitor with antifibrotic and immunomodulatory effects, was evaluated in two phase 3 trials (FIBONEER-IPF and FIBONEER-ILD) involving over 1100 IPF and 1176 PPF patients. At doses of 18 mg and 9 mg twice daily, nerandomilast significantly slowed forced vital capacity (FVC) decline compared to placebo over 52 weeks, including in patients already on antifibrotics like nintedanib. However, concomitant use with pirfenidone reduced nerandomilast plasma levels and efficacy. The most common adverse event was diarrhea, sometimes leading to discontinuation, especially when combined with nintedanib.<br /><br />Pulmonary rehabilitation (PR) was associated with improved survival in ILD patients over 5 years, based on analysis of prior randomized trials. PR improves lung function, symptoms, and health-related quality of life (HRQL), reinforcing its role in ILD care.<br /><br />In pulmonary sarcoidosis, a randomized trial found no superiority of high-dose prednisone (40 mg) over low-dose (20 mg) in reducing relapse or treatment failure. Both regimens had similar side effects and HRQL improvements. Additionally, a 24-week multicenter trial comparing methotrexate to prednisone as first-line therapy showed methotrexate’s noninferiority in improving FVC percentage predicted, suggesting it as a viable steroid-sparing alternative, with differing side effect profiles.<br /><br />Regarding ILAs—CT scan abnormalities suggesting early ILD in asymptomatic individuals—experts recommend defined evaluation and monitoring strategies. Screening chest CTs are advised for at-risk populations including smokers, connective tissue disease patients, and those with family histories of pulmonary fibrosis. Baseline symptom assessment and pulmonary function tests guide diagnosis; however, baseline lung biopsy or genetic tests (MUC5B, telomere length) are not recommended. Follow-up CT scans after 2-3 years are advised due to the substantial risk of progression (up to 46%), with some cases advancing to ILD.<br /><br />In summary, nerandomilast shows promise in slowing lung function decline in IPF and PPF; pulmonary rehabilitation may improve survival; lower-dose prednisone and methotrexate provide effective sarcoidosis treatment options; and careful monitoring of ILAs is crucial for early detection and management of progressive lung disease.

Keywords

Interstitial Lung Disease

Idiopathic Pulmonary Fibrosis

Progressive Pulmonary Fibrosis

Pulmonary Sarcoidosis

Interstitial Lung Abnormalities

Nerandomilast

Pulmonary Rehabilitation

Prednisone Dosage

Methotrexate Therapy

Lung Function Decline