Role of Steroids in Interstitial Lung Disease
Corticosteroids are not recommended for chronic management of idiopathic pulmonary fibrosis (IPF) but are appropriate first-line therapy for many non-IPF interstitial lung diseases, with treatment regimens tailored to the specific ILD subtype. 1
Different ILD Subtypes Require Different Steroid Approaches
Idiopathic Pulmonary Fibrosis (IPF)
- Steroids NOT recommended for chronic management
- Corticosteroids in IPF (especially in combination with azathioprine and N-acetyl-cysteine; "triple therapy") have been associated with increased mortality compared to placebo 2
- Antifibrotic therapy (pirfenidone, nintedanib) should be the cornerstone of treatment 1
- Exception: High-dose corticosteroids (methylprednisolone 1-2 mg/kg/day) are appropriate during acute exacerbations of IPF 1, 3
- Study showed no improvement in outcomes with higher doses of corticosteroids in acute exacerbation of IPF 3
Connective Tissue Disease-Associated ILD
- Steroid recommendations vary by specific disease:
Systemic Sclerosis (SSc)-ILD: Strong recommendation AGAINST glucocorticoids as first-line treatment 1
- Preferred therapy: Mycophenolate
- Alternative options: Tocilizumab, cyclophosphamide
Other SARD-ILDs (Myositis, Mixed Connective Tissue Disease, Rheumatoid Arthritis, Sjögren's): Short-term glucocorticoids (≤3 months) recommended 1
- Preferred therapy: Mycophenolate with short-term steroids
- Alternative options: Azathioprine, cyclophosphamide, JAK inhibitors (for myositis)
Sjögren's-Associated ILD
- Moderate-dose oral corticosteroids (up to 60 mg daily prednisone with slow taper over weeks-months) for moderate-severe disease 1
- Pulse-dose IV corticosteroids or high-dose oral prednisone (up to 60 mg daily) for rapidly progressive ILD or respiratory failure 1
- Steroid-sparing strategy: Add mycophenolate or azathioprine as first-line steroid-sparing agents 1
Dosing and Administration
Initial Dosing
- Moderate-severe disease: Prednisone 0.5-0.75 mg/kg/day (typically 40-60 mg daily) 1
- High-dose period typically lasts 2-4 weeks 1
- Acute exacerbations: Methylprednisolone 1-2 mg/kg/day 1, 3
Tapering Schedule
- Gradual taper over weeks to months based on clinical response 1
- Aim for lowest effective dose or complete discontinuation if possible 1
- For non-IPF ILD, higher doses of corticosteroids (>1 mg/kg prednisolone) may improve outcomes in acute exacerbations 3
Monitoring Response and Adverse Effects
Response Monitoring
- Pulmonary function tests every 3-6 months 1
- Clinical parameters (dyspnea, exercise tolerance) 1
- Radiographic findings 1
Adverse Effects to Monitor
- Hyperglycemia, hypertension, osteoporosis, adrenal suppression 1
- Increased risk of infections 1
- Psychiatric effects (mood changes, mania) 1
- Baseline steroid use may decrease efficacy of immune checkpoint inhibitors in patients with thoracic malignancies 1
Steroid-Resistant ILD Management
First-line Steroid-Sparing Agents
- Mycophenolate and azathioprine 1, 4
- For progressive disease despite steroids: Consider rituximab, cyclophosphamide, or nintedanib 1, 4
For IPF Specifically
Important Clinical Considerations
- Response is usually partial and transient, with few patients achieving complete remission 1
- Subjective improvement alone is not adequate to gauge response due to mood-enhancing effects of steroids 1
- Consider prophylaxis against Pneumocystis pneumonia for patients receiving prolonged corticosteroid therapy (≥20 mg methylprednisolone or equivalent for ≥4 weeks) 1
- Provide calcium and vitamin D supplementation for bone protection 1
Supportive Care
- Long-term oxygen therapy for patients with severe hypoxemia 1
- Pulmonary rehabilitation for patients with exercise limitation 1
- Annual influenza and pneumococcal vaccination 1
- Management of comorbidities 1
Remember that the mortality of acute exacerbation of ILD remains high (~50%) despite treatment, highlighting the need for prompt and effective management 1.