Essential Interstitial Lung Disease Management for the Primary Care Provider

Abstract

Interstitial lung diseases (ILDs) encompass a diverse group of disorders characterized by inflammation and fibrosis of the lung parenchyma. Despite their classification as rare, increasing evidence suggests ILDs are more prevalent than previously thought. Patients often present with respiratory symptoms such as exertional dyspnea, persistent cough, and fatigue. However, asymptomatic patients with incidental findings on imaging (e.g., interstitial lung abnormalities) are also common. Diagnosis relies on high-resolution CT (HRCT), pulmonary function tests, and detailed clinical evaluation. Respirology consultation is important for comprehensive management. The evolving ILD nomenclature, including progressive pulmonary fibrosis, aids in disease characterization and treatment planning. Management strategies include corticosteroids and steroid-sparing agents for inflammatory subtypes, while antifibrotic therapies (nintedanib, pirfenidone) are used for fibrotic and progressive disease. Non-pharmacological interventions, including pulmonary rehabilitation, smoking cessation, and vaccination, are critical for improving patient outcomes. Primary care providers play a pivotal role in early disease recognition, facilitating diagnostic testing, managing comorbidities, and coordinating specialist care. This review highlights the importance of timely diagnosis, evolving classifications, and emerging therapies, offering a collaborative framework for optimizing ILD care and outcomes.