Prognosis and Treatment for Advanced Interstitial Lung Disease (ILD)

Disease Progression and Treatment Approaches

Disease progression is defined by decline in forced vital capacity (FVC) of ≥10% of predicted value, or decline of 5-10% with worsening respiratory symptoms or increased fibrosis on HRCT within 24 months, as defined by the American Thoracic Society 1
Rapidly progressive ILD (RP-ILD) represents a particularly aggressive subtype with rapid progression from baseline oxygen requirement to high oxygen needs or intubation within days to weeks, according to the American College of Rheumatology 1
Immunomodulatory therapy is first-line treatment for Connective Tissue Disease-Associated ILD, with options including mycophenolate, azathioprine, rituximab, and cyclophosphamide, as recommended by the European League Against Rheumatism 1
For systemic sclerosis-associated ILD, glucocorticoids are strongly recommended against as first-line treatment, according to the American College of Rheumatology 1
For other SARD-ILD (systemic autoimmune rheumatic disease-ILD), glucocorticoids are conditionally recommended as first-line treatment, as suggested by the European League Against Rheumatism 1

Chronic cough affects up to 80% of IPF patients and significantly impairs quality of life, according to the American Thoracic Society 2
First-line options for refractory cough in advanced ILD include gabapentin therapy and multimodality speech pathology therapy (including cough suppression techniques), as recommended by the American College of Chest Physicians 3, 2
For intractable cough with substantial impact on quality of life when alternative treatments have failed, opiates should be considered for symptom control in a palliative care setting, according to the American College of Chest Physicians 3, 4
When using opiates for symptom control, regular reassessment of benefits versus risks is essential, as suggested by the American Thoracic Society 2

Regular pulmonary function tests, including spirometry and diffusing capacity for carbon monoxide (DLCO), should be performed at least every 6 months in higher-risk patients and yearly in others, as recommended by the European Respiratory Society 6
Functional assessment should include 6-minute walk distance test, which is widely recognized, simple, non-invasive, and reproducible, according to the American Thoracic Society 6