What is the treatment approach for Rheumatoid Arthritis (RA) related Interstitial Lung Disease (ILD)?

From the Guidelines

The treatment approach for Rheumatoid Arthritis (RA) related Interstitial Lung Disease (ILD) typically involves immunosuppressive therapy, with glucocorticoids conditionally recommended as a first-line ILD treatment for people with SARD-ILD other than SSc-ILD, according to the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1. The guideline conditionally recommends mycophenolate, azathioprine, rituximab, and cyclophosphamide as first-line ILD treatment options for people with SARD-ILD 1.

• For people with RA-ILD, the Panel was not able to come to consensus on whether to recommend nintedanib as a first-line ILD treatment option 1.
• Some panelists would consider using nintedanib for patients with a UIP pattern of ILD irrespective of SARD 1.
• The guideline emphasizes co-management of people with SARD-ILD by rheumatologists and pulmonologists, and recommends against the use of methotrexate, leflunomide, TNFi, and abatacept for SARD-ILD treatment 1.
• Treatment should be individualized based on ILD pattern, disease severity, and progression rate, with supportive care including pulmonary rehabilitation, oxygen therapy when indicated, vaccination against respiratory pathogens, and smoking cessation being essential components of management 1.
• Regular monitoring with pulmonary function tests every 3-6 months and high-resolution CT scans annually or when clinically indicated is necessary to assess treatment response and disease progression 1. The 2023 ACR/CHEST guideline provides recommendations for ILD treatment decisions frequently faced in clinical practice, and emphasizes the importance of shared decision-making and co-management by rheumatologists and pulmonologists 1.

From the Research

Treatment Approach for Rheumatoid Arthritis (RA) related Interstitial Lung Disease (ILD)

The treatment approach for RA-related ILD involves a combination of medications and considerations of various factors.

• The treatment requires immediate cessation of methotrexate (MTX) and commencement of glucocorticoids in cases of MTX-pneumonitis 2.
• For RA-ILD, treatment options include glucocorticoids, conventional disease-modifying antirheumatic drugs (DMARDs) like MTX or leflunomide, as well as biologic DMARDs 2.
• The 2023 American College of Rheumatology/American College of Chest Physicians guideline conditionally recommends mycophenolate, azathioprine, and rituximab for first-line RA-ILD therapy, with cyclophosphamide and short-term glucocorticoids as additional options 3.
• Antifibrotics might be particularly effective for patients with RA-ILD and the usual interstitial pneumonia pattern (RA-UIP) 3.
• Immunosuppression with mycophenolate, azathioprine, or rituximab has been associated with an improved trajectory in forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (Dlco) compared to the pretreatment pulmonary function trajectory 4.

Considerations for Treatment

• Treatment decisions should consider articular activity, ILD activity, comorbidities, and potential for infection 3.
• The use of MTX, leflunomide, and tacrolimus does not increase the risk of ILD progression, except for leflunomide in patients with severe ILD 5.
• Older age, male sex, shorter RA duration, higher RA disease activity, and extensive disease at baseline are independently associated with ILD progression 5.
• The appropriate use of conventional synthetic disease-modifying antirheumatic drugs considering RA disease activity and ILD severity is important for the management of RA-associated ILD 5.

Current Research and Recommendations

• Research interest in RA-ILD has increased in recent years, with some studies suggesting that RA-ILD prevalence may be increasing 6.
• Novel RA-ILD risk factors include the MUC5B promotor variant, articular disease activity, autoantibodies, and biomarkers of damaged pulmonary parenchyma 6.
• Treatment should focus on controlling RA disease activity, which emerging data suggest may reduce RA-ILD risk 6.
• Immunomodulatory and antifibrotic drugs may also treat RA-ILD 6.