Treatment of Chronic Interstitial Lung Disease

Introduction to ILD Treatment

The American College of Rheumatology recommends antifibrotic therapy with nintedanib or pirfenidone as first-line treatment for idiopathic pulmonary fibrosis (IPF), while immunomodulatory therapy with mycophenolate, azathioprine, rituximab, or cyclophosphamide is recommended as first-line treatment for connective tissue disease-associated ILD (CTD-ILD) 1, 2

For connective tissue disease-associated ILD (CTD-ILD), immunomodulatory therapy is first-line treatment, with mycophenolate, azathioprine, rituximab, and cyclophosphamide all conditionally recommended as first-line options 1, 2, 3
For systemic sclerosis-associated ILD (SSc-ILD), glucocorticoids are strongly recommended against as first-line treatment due to increased risk of scleroderma renal crisis 1, 2, 3
For SSc-ILD specifically, nintedanib is conditionally recommended as a first-line option, and tocilizumab is conditionally recommended for both SSc-ILD and MCTD-ILD 1, 2

Disease progression is defined as FVC decline ≥10% of predicted value, or FVC decline 5-10% with worsening respiratory symptoms or increased fibrosis on HRCT, within 24 months 3
For SSc-ILD progression, mycophenolate, rituximab, cyclophosphamide, and nintedanib are conditionally recommended 1
For RA-ILD progression, adding pirfenidone is conditionally recommended 1
For IIM-ILD progression, calcineurin inhibitors (CNIs) and JAK inhibitors are conditionally recommended 4

For refractory chronic cough in ILD, gabapentin (neuromodulator) and multimodality speech pathology therapy are suggested as first-line options 5
Opiates should be considered for intractable cough with substantial impact on quality of life when alternative treatments have failed, with regular reassessment of benefits versus risks 5, 3

Pulmonary function tests (spirometry and DLCO) should be performed at least every 6 months in higher-risk patients and yearly in others 3
Six-minute walk distance test should be included in functional assessment as it is simple, non-invasive, and reproducible 3

Referral for lung transplant should be considered for patients with advanced ILD before the patient deteriorates and is no longer eligible 4

Glucocorticoids should not be used as first-line treatment in SSc-ILD due to risk of scleroderma renal crisis 1, 2, 3
Inhaled corticosteroids should not be routinely prescribed for chronic cough in pulmonary sarcoidosis 5
Nintedanib or pirfenidone should not be added to mycophenolate in SARD-ILD patients without evidence of ILD progression 1, 2