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Overview of Interstitial Lung Disease (ILD)

• ILD refers to a group of lung diseases that affect the interstitium of the lung, resulting in damage to septa, alveolar epithelial cells, respiratory bronchioles, capillary endothelial cells, and pulmonary vasculature.
• ILD can present with different histopathology patterns, including granulomatous lung disease, non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), and usual interstitial pneumonia (UIP).
• Hypersensitivity pneumonitis is an inflammatory disease caused by exposure to organic dust and can convert to fibrosis. The most important intervention is removing antigenic exposure and treating with immunosuppression if symptomatic, extensive, or progressive.
• Sarcoidosis is an inflammatory disease that can affect almost any part of the body, but the lungs and lymph nodes are the most common site of disease. Treatment with corticosteroids or other immunosuppression medications is recommended for bothersome, progressive, or 'dangerous' disease.
• Drug-related ILD can be caused by chemotherapy agents, amiodarone, and long-term nitrofurantoin. The most important intervention is stopping the medication, and treatment with immunosuppression is recommended if symptomatic, extensive, or progressive.
• Connective tissue related ILD can be triggered by scleroderma, rheumatoid arthritis, dermatomyositis/polymyositis, Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus (SLE). Treatment with immunosuppression is recommended if symptomatic, extensive, or progressive.
• IPF is a common ILD disease of older age that mostly affects the lower and peripheral aspects of the lung, with fine peripheral lines and honeycomb cysts. Treatment with anti-fibrotic medications is indicated for extensive and progressive ILD.
• All fibrotic ILDs do not start with inflammation, and IPF does not have a typical inflammatory prodrome phase.
• Pulmonary function testing results can help diagnose and monitor ILD, including forced vital capacity (FVC), diffusing capacity for carbon monoxide (DLCO), and the ratio of forced expiratory volume in 1 second (FEV1) to FVC.
• ILD can have a significant impact on quality of life and mortality, and early diagnosis and treatment are crucial.
• ILD can be challenging to diagnose and manage, and a multidisciplinary approach involving pulmonologists, radiologists, pathologists, and rheumatologists is often necessary.
• ILD research is ongoing, and new therapies are being developed to improve outcomes for patients with ILD.