From the Guidelines
The treatment approach for suspected mild or early interstitial lung changes should focus on identifying and addressing the underlying cause while preventing disease progression, with a multidisciplinary approach being essential for optimal care 1. The underlying cause should be identified and eliminated if possible, such as removing exposure to environmental or occupational triggers, discontinuing medications that may be causing lung damage, or treating underlying autoimmune conditions.
- Corticosteroids are often the first-line medication, typically starting with prednisone at 0.5-1 mg/kg/day (usually 40-60 mg daily) for 4-12 weeks, followed by a slow taper based on clinical response.
- For steroid-resistant cases or to reduce steroid requirements, immunosuppressive agents like mycophenolate mofetil (1-1.5 g twice daily), azathioprine (2-3 mg/kg/day), or cyclophosphamide may be added.
- Antifibrotic medications such as pirfenidone (801 mg three times daily) or nintedanib (150 mg twice daily) may be considered for idiopathic pulmonary fibrosis, as suggested by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1. Supportive care is essential, including:
- Supplemental oxygen if oxygen saturation falls below 88%
- Pulmonary rehabilitation to maintain exercise capacity
- Vaccination against respiratory infections
- Smoking cessation Regular monitoring with pulmonary function tests every 3-6 months, chest imaging, and oxygen saturation measurements helps assess disease progression and treatment response, with the optimal interval for follow-up HRCT to determine disease progression being unknown, but limited data suggesting that in patients with systemic sclerosis and stable pulmonary function, repeated chest HRCT within 12–24 months from baseline could be useful to promptly detect progression and possibly influence prognosis 1. Early intervention is crucial as established fibrosis is generally irreversible, and the goal is to preserve lung function and prevent further deterioration. A multidisciplinary approach, including rheumatologists and pulmonologists, is necessary to ensure optimal coordination and quality of patient care, as emphasized in the practical guidance for the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease 1.
From the Research
Treatment Approach for Suspected Mild or Early Interstitial Lung Changes
The treatment approach for suspected mild or early interstitial lung changes involves a multidisciplinary discussion to evaluate features that indicate an increased risk of progression 2.
- For patients with idiopathic pulmonary fibrosis (IPF), antifibrotic therapy with nintedanib or pirfenidone may be used to slow annual forced vital capacity (FVC) decline by approximately 44% to 57% 3.
- For connective tissue disease-associated interstitial lung disease, immunomodulatory therapy such as tocilizumab, rituximab, and mycophenolate mofetil may be used to slow decline or even improve FVC at 12-month follow-up 3.
- Structured exercise therapy and oxygen therapy may also be used to reduce symptoms and improve quality of life in individuals with interstitial lung disease 3.
- In patients with interstitial lung disease who do not respond to steroid therapy, immunosuppressive drugs such as azathioprine or cyclophosphamide may be considered 4.
Monitoring and Surveillance
Patients with interstitial lung abnormalities (ILAs) should be monitored for progression to full-blown interstitial lung disease (ILD) 5.
- A multidisciplinary discussion should evaluate features that indicate an increased risk of progression, and serial monitoring is recommended for patients with features that indicate an increased risk of progression 2.
- For patients without imaging or clinical features that indicate an increased risk of progression, reactive monitoring is recommended 2.
Disease-Specific Management
If ILD is subsequently diagnosed, the management is disease-specific 2.