What are the management options for interstitial lung disease?

Management Options for Interstitial Lung Disease

Mycophenolate is the preferred first-line treatment for most types of systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD), with specific treatment algorithms varying by disease subtype and disease progression. 1

First-Line Treatment Options for SARD-ILD

Preferred First-Line Medications

• Mycophenolate is conditionally recommended as the preferred first-line therapy across all SARD-ILD subtypes 1
• Azathioprine is a conditionally recommended alternative first-line option for many SARD-ILD subtypes except systemic sclerosis (SSc) 1
• Rituximab is conditionally recommended across all SARD-ILD subtypes 1
• Cyclophosphamide is conditionally recommended as an additional option for most SARD-ILD subtypes 1

Disease-Specific First-Line Options

• For SSc-ILD: Tocilizumab and nintedanib are conditionally recommended as first-line options 1
• For idiopathic inflammatory myopathy (IIM)-ILD: JAK inhibitors and calcineurin inhibitors (CNIs) are conditionally recommended 1
• For rheumatoid arthritis (RA)-ILD: No consensus was reached regarding nintedanib as first-line therapy 1

Medications to Avoid as First-Line Therapy

• Glucocorticoids are strongly recommended against for SSc-ILD due to risk of scleroderma renal crisis 1
• Glucocorticoids may be conditionally recommended for other SARD-ILD subtypes, but should be used cautiously 1
• Leflunomide, methotrexate, TNF inhibitors, and abatacept are conditionally recommended against as first-line options 1
• IVIG and plasma exchange are conditionally recommended against as first-line options 1

Management of Progressive SARD-ILD

When patients show progression despite first-line therapy:

• Mycophenolate, rituximab, cyclophosphamide, and nintedanib are conditionally recommended treatment options 1
• Long-term glucocorticoids are strongly recommended against in SSc-ILD and conditionally recommended against in other SARD-ILD subtypes 1
• For RA-ILD progression: Adding pirfenidone is conditionally recommended 1, 2
• For SSc-ILD, MCTD-ILD, or RA-ILD progression: Tocilizumab is conditionally recommended 1
• For IIM-ILD progression: Calcineurin inhibitors and JAK inhibitors are conditionally recommended 1
• For SSc-ILD progression: Referral for stem cell transplantation and/or lung transplantation is conditionally recommended 1

Management of Rapidly Progressive ILD (RP-ILD)

• Pulse intravenous methylprednisolone is conditionally recommended as first-line treatment 1
• Rituximab, cyclophosphamide, IVIG, mycophenolate, CNIs, and JAK inhibitors are conditionally recommended as first-line options 1
• Upfront combination therapy is conditionally recommended over monotherapy (triple therapy for those with confirmed or suspected MDA-5) 1
• Early referral for lung transplantation is conditionally recommended over later referral 1

Monitoring and Follow-up

• Regular monitoring with pulmonary function tests is essential, as a 5% decline in forced vital capacity (FVC) over 12 months is associated with approximately 2-fold increase in mortality 3
• High-resolution computed tomography (HRCT) is approximately 91% sensitive and 71% specific for diagnosing ILD subtypes 3
• Multidisciplinary team approach involving pulmonologists and rheumatologists is recommended for optimal management 1

Additional Supportive Measures

• Structured exercise therapy reduces symptoms and improves 6-minute walk test distance 3
• Oxygen therapy reduces symptoms and improves quality of life in patients who desaturate below 88% on a 6-minute walk test 3
• Lung transplantation should be considered for end-stage ILD, with median survival of 5.2-6.7 years post-transplant compared to less than 2 years without transplant 3, 4

Common Pitfalls and Caveats

• Avoid using glucocorticoids in SSc-ILD patients due to risk of scleroderma renal crisis, particularly at doses >15mg/day of prednisone equivalent 1
• Treatment decisions should consider disease subtype, severity, progression rate, and fibrotic pattern on imaging 1
• Cyclophosphamide is typically not used in combination with other therapies, unlike other agents which may be used individually or in combination 1
• For patients with pulmonary hypertension associated with ILD, specific pulmonary vasodilator therapy may be needed 3, 4
• Early recognition and treatment are crucial, as many forms of ILD can progress to irreversible fibrosis if not managed appropriately 1, 5