From the Guidelines
The initial treatment for chronic interstitial lung disease (ILD) should be guided by the specific type of ILD, with mycophenolate, azathioprine, rituximab, and cyclophosphamide conditionally recommended as first-line ILD treatment options for people with SARD-ILD other than SSc-ILD, as stated in the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1. When considering treatment for chronic interstitial lung disease, it is essential to differentiate between various forms of the disease, as they respond differently to therapy.
- For people with SSc-ILD, nintedanib is conditionally recommended as a first-line ILD treatment option, whereas for those with SjD-ILD, IIM-ILD, and MCTD-ILD, nintedanib is conditionally recommended against as a first-line treatment 1.
- Glucocorticoids are conditionally recommended as a first-line ILD treatment for people with SARD-ILD other than SSc-ILD, but strongly recommended against for those with SSc-ILD 1.
- Tocilizumab is conditionally recommended as a first-line ILD treatment option for people with SSc-ILD and MCTD-ILD 1.
- JAK inhibitors (JAKi) and calcineurin inhibitors (CNIs) are conditionally recommended as first-line ILD treatment options for people with IIM-ILD, but conditionally recommended against for those with SARD-ILD other than IIM-ILD 1. The treatment approach should prioritize reducing inflammation, preventing immune-mediated damage, and targeting the scarring process in the lungs, while also considering the specific type of ILD and the patient's overall health status.
- Supportive care, including supplemental oxygen, pulmonary rehabilitation, vaccination against respiratory infections, and management of comorbidities, is essential to improve exercise capacity, quality of life, and overall outcomes for patients with chronic interstitial lung disease.
From the Research
Initial Treatment for Chronic Interstitial Lung Disease
The initial treatment for chronic interstitial lung disease (ILD) depends on the underlying cause and severity of the disease.
- For idiopathic pulmonary fibrosis (IPF), first-line therapy includes antifibrotic medications such as nintedanib or pirfenidone, which can slow the decline in lung function 2.
- For ILD due to connective tissue disease, immunomodulatory therapy such as mycophenolate mofetil may be used to slow the decline in lung function 2, 3, 4.
- In patients with rheumatoid arthritis-associated ILD, treatment with mycophenolate mofetil, azathioprine, or rituximab may improve lung function and slow disease progression 3, 4.
- Antifibrotic medications such as nintedanib and pirfenidone may also be considered for patients with progressive pulmonary fibrosis of any cause, including those with connective tissue disease-associated ILD 2, 4.
Additional Therapies
Additional therapies that may be used to manage symptoms and improve quality of life in patients with ILD include:
- Oxygen therapy to reduce symptoms and improve quality of life in patients who desaturate below 88% on a 6-minute walk test 2.
- Structured exercise therapy to reduce symptoms and improve 6-minute walk test distance 2.
- Lung transplantation may be considered for patients with advanced ILD who have not responded to other treatments 2.
- Treatment with inhaled treprostinil may improve walking distance and respiratory symptoms in patients with pulmonary hypertension due to ILD 2.