What is the recommended steroid treatment regimen for patients with Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD)?

Steroid Treatment Regimens for Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD)

For patients with CTD-ILD, pulse dose IV methylprednisolone (1000 mg daily for 3 days) followed by moderate-dose oral prednisone (up to 60 mg daily) with a slow taper is recommended for rapidly progressive disease or acute respiratory failure, while avoiding long-term glucocorticoids in systemic sclerosis-ILD. 1

Initial Treatment Approach Based on Disease Type and Severity

Rapidly Progressive ILD or Acute Respiratory Failure

• For patients with rapidly progressive CTD-ILD or acute respiratory failure, pulse dose IV methylprednisolone (1000 mg daily for 3 days) is recommended as first-line treatment 2, 1
• Follow pulse therapy with moderate-to-high dose oral prednisone (up to 60 mg daily) with a slow taper over weeks to months 1
• Alternative etiologies, such as infections or lymphoproliferative disorders, must be considered before initiating high-dose steroids 2

Disease-Specific Steroid Recommendations

• Systemic Sclerosis-ILD (SSc-ILD): Strongly recommended AGAINST using glucocorticoids as first-line treatment due to risk of scleroderma renal crisis 2
• Inflammatory Myopathy-ILD (IIM-ILD): Short-term glucocorticoids are recommended as part of initial therapy 2
• Mixed Connective Tissue Disease-ILD (MCTD-ILD): Short-term glucocorticoids are recommended, but should be used cautiously in patients with SSc phenotype who may be at increased risk of renal crisis 2
• Rheumatoid Arthritis-ILD (RA-ILD): Short-term glucocorticoids are recommended as part of initial therapy 2
• Sjögren's-ILD: Short-term glucocorticoids are recommended as part of initial therapy 2

Steroid Dosing and Administration

Pulse Therapy for Severe/Rapidly Progressive Disease

• IV methylprednisolone 1000 mg daily for 3 days 1, 3
• Two courses of pulse dose methylprednisolone therapy have shown multidimensional efficacy in a cohort of CTD-ILD patients 3

Oral Steroid Regimens

• Initial oral prednisone: 5-60 mg per day depending on disease severity 4
• Take before 9 am to minimize adrenal suppression 4
• Administer with food or milk to reduce gastric irritation 4
• Maintain or adjust initial dosage until satisfactory response is noted 4

Tapering Strategies

• After favorable response, decrease initial dose in small increments at appropriate intervals 4
• Aim for lowest dosage that maintains adequate clinical response 4
• If long-term therapy is required, consider alternate-day therapy (twice the usual daily dose every other morning) to minimize side effects 4
• Avoid abrupt withdrawal of therapy 4

Steroid-Sparing Agents (First-Line Therapies)

Preferred First-Line Agents

• Mycophenolate mofetil is conditionally recommended as the preferred first-line therapy for all types of SARD-ILD 2
• Azathioprine is recommended as a first-line option for myositis-ILD, MCTD-ILD, RA-ILD, and Sjögren's-ILD 2
• Rituximab is recommended as a first-line option for all types of SARD-ILD 2

Disease-Specific Additional Options

• SSc-ILD: Tocilizumab, cyclophosphamide 2
• Myositis-ILD: JAK inhibitors, calcineurin inhibitors 2
• MCTD-ILD: Tocilizumab, cyclophosphamide 2
• RA-ILD: Tocilizumab, cyclophosphamide 2
• Sjögren's-ILD: Cyclophosphamide 2

Management of Treatment Failure or Disease Progression

• For patients with SSc-ILD progression, strongly recommended AGAINST using long-term glucocorticoids 2
• For other SARD-ILD progression, conditionally recommended AGAINST using long-term glucocorticoids 2
• For SARD-ILD progression despite first-line treatment, conditionally recommend mycophenolate, rituximab, cyclophosphamide, and nintedanib as treatment options 2
• For rapidly progressive or exacerbating Sjögren's-ILD, consider rituximab or cyclophosphamide in addition to high-dose corticosteroids 2

Monitoring During Treatment

• Daily assessment of respiratory status and oxygen requirements during inpatient treatment 1
• Monitor for steroid-related complications, such as hyperglycemia and hypertension 1
• Serial pulmonary function tests as clinically indicated 1
• Close monitoring of pulmonary function tests every 3-6 months is recommended, especially in the first 1-2 years after treatment initiation 1

Potential Pitfalls and Caveats

• High-dose steroids in SSc-ILD can trigger scleroderma renal crisis, particularly at prednisone doses >15 mg daily 2
• Be aware of potential drug-induced lung disease from medications used to treat CTD-ILD, including TNF-alpha inhibitors, sulfasalazine, cyclophosphamide, rituximab, leflunomide, methotrexate, and sulfonamides 2
• Constant monitoring is needed for dosage adjustments based on clinical status changes, disease remissions/exacerbations, and patient exposure to stressful situations 4
• Pneumocystis jirovecii prophylaxis should be provided when cyclophosphamide is used 2