Clinical updates on interstitial lung disease

A review of the evolving therapeutic landscape of interstitial lung diseases, exploring the expanding role of precision medicine, multidisciplinary care, and artificial intelligence

Interstitial lung diseases (ILDs) represent a diverse group of lung disorders characterised by varying degrees of inflammation and fibrosis of the lung interstitium. The delicate tissue surrounding the lung’s alveoli becomes progressively fibrosed through prolonged repetitive injury, inflammation, and collagen deposition. This prevents the normal architecture of the lungs from performing gas exchange as intended, reducing oxygen delivery to vital organs and resulting in the onset of progressive, debilitating symptoms of breathlessness and cough. Over time, the lungs become less elastic as fibrosis continues, leading to reduced oxygen availability to tissues and ultimately progressing to respiratory failure.

ILDs consist of idiopathic interstitial pneumonias with the commonest being idiopathic pulmonary fibrosis (IPF), ILDs associated with connective tissue diseases (CTD-ILD), which are autoimmune-modulated, ILDs triggered by environmental and occupational exposures (for example, bird and mould exposure and asbestos), drug and radiation-induced ILDs, and sarcoidosis (Figure 1). IPF and progressive fibrotic non-IPF ILDs, termed progressive pulmonary fibrosis (PPF), are associated with the highest morbidity and mortality. Our understanding of the pathophysiology of disease and disease burden has evolved, and with it have come newer approaches to diagnosis and management of ILD.


FIGURE 1: Types of interstitial lung disease (Abbreviations: ILD – interstitial lung disease)
At present, the current evidence-based treatment focuses primarily on antifibrotic therapy, with pirfenidone and nintedanib forming the standard of care for IPF and selected cases of PPF. However, these therapies slow down, but do not halt, disease progression and are often limited by tolerability issues. This clinical update reviews the evolving therapeutic landscape of ILDs, exploring the expanding role of precision medicine, multidisciplinary care, and artificial intelligence in advancing both diagnostic accuracy and individualised treatment strategies.