What is the recommended steroid tapering regimen for a patient with an acute exacerbation of interstitial lung disease (ILD) who is currently on high-dose corticosteroids, such as prednisone?

Steroid Tapering for Acute Exacerbation of ILD

For acute exacerbation of ILD, initiate high-dose IV methylprednisolone (1000 mg daily for 3 days) followed by oral prednisone 0.5-1.0 mg/kg daily (typically 40-60 mg), then taper slowly over 2-4 months while simultaneously starting a steroid-sparing immunosuppressant like mycophenolate mofetil. 1

Initial High-Dose Steroid Phase

Pulse Therapy:

• Start with IV methylprednisolone 1000 mg daily for 3 consecutive days for rapidly progressive disease or acute respiratory failure 1
• Alternatively, use IV methylprednisolone 1-2 mg/kg/day for severe cases 2
• Rule out infections and lymphoproliferative disorders before initiating high-dose steroids 2, 1

Transition to Oral Steroids:

• After pulse therapy, switch to oral prednisone 0.5-1.0 mg/kg daily (typically 40-60 mg for a 70 kg patient) 1
• Higher doses (>1.0 mg/kg) show improved outcomes in non-IPF ILD but not in IPF 3

Tapering Schedule

First Month (Days 4-30):

• Maintain prednisone at initial dose (40-60 mg daily) for 2-4 weeks if clinical improvement occurs 2, 1
• Monitor daily for respiratory status, oxygen requirements, and steroid complications 1

Months 2-3:

• If symptoms improve to grade ≤2, begin slow taper by reducing 5-10 mg every 1-2 weeks 2
• Target dose of 20-30 mg daily by end of month 2 1
• Continue reducing by 5 mg every 2 weeks 2

Months 3-4 and Beyond:

• Taper to 10-20 mg daily by month 3 2, 1
• Further reduce by 2.5-5 mg every 2-4 weeks 2
• Goal is to reach maintenance dose of 5-10 mg daily or discontinue entirely by 4-6 months 1
• Total taper duration should be >2 months for severe cases 2

Critical Disease-Specific Modifications

Systemic Sclerosis-ILD:

• Avoid glucocorticoids entirely if possible due to high risk of scleroderma renal crisis, particularly at doses >15 mg daily 1
• If steroids absolutely necessary, use lowest effective dose with close renal monitoring 1
• Never use long-term glucocorticoids in SSc-ILD 1

Non-IPF ILD (Sjögren's, RA-ILD, Myositis-ILD, MCTD-ILD):

• Higher steroid doses (>1.0 mg/kg) show mortality benefit 3
• Standard tapering protocol applies 1

IPF:

• High-dose steroids do not improve outcomes 3
• Consider lower initial doses (0.5 mg/kg) or avoid steroids altogether 3

Mandatory Concurrent Steroid-Sparing Therapy

Initiate Immediately (Within First Week):

• Mycophenolate mofetil 500-1000 mg twice daily, target 1500 mg twice daily - preferred first-line agent for all CTD-ILD types 1
• Alternative: Azathioprine for myositis-ILD, MCTD-ILD, RA-ILD, or Sjögren's-ILD 1
• For refractory cases: Rituximab (1000 mg IV on days 1 and 15) or cyclophosphamide (500-750 mg/m² IV every 4 weeks) 1

Rationale:

• Early immunosuppression prevents irreversible fibrosis 1
• Allows faster steroid taper and reduces long-term steroid toxicity 1
• Combination therapy superior to monotherapy for rapidly progressive disease 1

Essential Supportive Measures

Infection Prophylaxis:

• Pneumocystis jirovecii prophylaxis (trimethoprim-sulfamethoxazole) for patients receiving ≥20 mg prednisone equivalent for ≥4 weeks 2, 1
• Proton pump inhibitor for GI prophylaxis 2

Bone Protection:

• Calcium and vitamin D supplementation with prolonged steroid use 2
• Consider bisphosphonate if anticipated steroid duration >3 months 1

Monitoring:

• Baseline: glucose, blood pressure, bone density, ophthalmologic exam, TB and hepatitis B screening 1
• During treatment: CBC with differential and liver function tests 2-3 weeks after starting immunosuppression, then every 3 months 1
• Serial pulmonary function tests every 3-6 months 1

Critical Pitfalls to Avoid

Do Not:

• Use long-term glucocorticoids (>3-6 months) as maintenance monotherapy - associated with poor outcomes and toxicity 1
• Inappropriately prolong steroids after clinical improvement - standardize taper protocols 2
• Taper too rapidly if clinical worsening occurs - return to previous effective dose 2
• Use high-dose steroids in SSc-ILD without extreme caution 1

If Disease Progresses During Taper:

• Return to last effective steroid dose 2
• Add or escalate steroid-sparing immunosuppression rather than increasing steroids long-term 1
• Consider adding nintedanib for progressive fibrotic ILD (except SSc-ILD where it may be first-line) 1
• Consider rituximab or cyclophosphamide for refractory cases 2, 1

When to Abandon Steroid Taper:

• Mechanical ventilation required within 3 days of hospitalization - poor prognostic sign 3
• Consider lung transplantation evaluation if FVC <50% predicted or DLCO <40% predicted despite maximal therapy 1