From the Guidelines
Empirical steroid treatment for interstitial lung disease (ILD) is appropriate when there is high clinical suspicion for inflammatory ILD subtypes, particularly in acute presentations with rapid deterioration, or when specific inflammatory patterns are suspected based on clinical and radiographic findings, as supported by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of ILD in people with systemic autoimmune rheumatic diseases 1.
Key Considerations
- The decision to initiate empirical steroid treatment should be based on a thorough evaluation of the patient's clinical presentation, radiographic findings, and pulmonary function tests.
- Prednisone is typically started 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 1.
- For acute, severe presentations, methylprednisolone 500-1000 mg IV daily for 3 days may be used before transitioning to oral therapy.
- Before initiating steroids, it's essential to rule out infection with appropriate cultures and consider obtaining a high-resolution CT scan.
- Patients should be monitored for clinical improvement within 2-4 weeks, with pulmonary function tests at baseline and follow-up.
Steroid-Responsive ILDs
- Cryptogenic organizing pneumonia
- Nonspecific interstitial pneumonia
- Hypersensitivity pneumonitis
- Sarcoidosis
Important Considerations
- Prophylaxis against Pneumocystis pneumonia should be considered for patients on prolonged high-dose therapy.
- Bone health should be monitored with calcium and vitamin D supplementation.
- The 2023 ACR/CHEST guideline conditionally recommends glucocorticoids as a first-line ILD treatment for people with SARD-ILD other than SSc-ILD 1.
From the Research
Empirical Treatment of ILD Patients with Steroids
- The use of steroids in the treatment of interstitial lung disease (ILD) is a topic of ongoing research and debate 2, 3, 4, 5, 6.
- According to a study published in 2024, steroid therapy may be associated with increased risk of inpatient mortality or transplantation in patients with acute exacerbation of fibrotic interstitial lung disease (AE-FILD) 6.
- However, another study published in 2012 found that a combination of low-dose steroids with cyclophosphamide was effective in treating interstitial lung disease, especially in active disease 4.
- A systematic review and meta-analysis published in 2024 found that mycophenolate mofetil (MMF) and azathioprine (AZA) may support preservation of pulmonary function in ILD, but the evidence was of very low certainty 5.
- A study published in 2019 found that AZA treatment was associated with improved forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) in patients with myositis-related ILD, and that patients treated with AZA received a lower prednisone dose than those treated with MMF 3.
Specific Patient Populations
- In patients with connective tissue disease-associated ILD, immunomodulatory therapy such as tocilizumab, rituximab, and mycophenolate mofetil may slow decline or even improve FVC at 12-month follow-up 2.
- In patients with idiopathic pulmonary fibrosis (IPF), antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% 2.
- In patients with AE-FILD, steroid use appeared to be associated with increased risk of inpatient mortality or transplantation, but may be beneficial in non-IPF patients 6.
Treatment Considerations
- The decision to use steroids in the treatment of ILD should be made on a case-by-case basis, taking into account the individual patient's disease severity, comorbidities, and other factors 2, 3, 4, 5, 6.
- Clinicians should consider other precipitating factors for exacerbations and use steroids judiciously 6.
- Further prospective trials are needed to determine the role of corticosteroids in AE-FILD and other forms of ILD 6.