From the Guidelines
Rheumatoid Arthritis (RA) associated Interstitial Lung Disease (ILD) trials are limited, but available options include nintedanib and pirfenidone as anti-fibrotic therapies.
Available Trials
- Nintedanib has been shown to slow down disease progress over time in patients with RA-ILD 1
- Pirfenidone's evidence is not sufficient due to the early termination of the TRAIL1 study, but it is still considered a therapeutic option for the management of progressive RA-ILD despite first-line treatment 1
Treatment Approach
- The approach to treating RA-ILD is traditionally integrated and multidisciplinary, involving both rheumatologists and pulmonologists 1
- Treatment with immunosuppressive agents is generally used regardless of the pattern of fibrosis, and patients are typically evaluated concurrently in a pulmonary and rheumatology consultation 1
- The 2023 ACR guidelines conditionally recommend adding the anti-fibrotic treatment nintedanib or pirfenidone as therapeutic options for the management of progressive RA-ILD despite first-line treatment 1
Key Considerations
- The clinical examination with auscultation are useful options for detecting Velcro-crackles, and risk factors must be considered to determine which patients are more likely to develop ILD 1
- Short-term PFTs and HRCT should be considered to determine the rate of progression, and patients with moderate-to-severe ILD at baseline or progressive disease must have more frequent PFTs 1
- The Patient Panel agreed to tolerate adverse effects for crucial treatments, but gastrointestinal adverse effects could significantly impact QoL 1
From the Research
Overview of Rheumatoid Arthritis-Associated Interstitial Lung Disease (RA-ILD) Trials
- There are several trials available for RA-ILD, including those investigating the efficacy of various medications such as mycophenolate, azathioprine, rituximab, and nintedanib 2, 3.
- The Treatment for Rheumatoid Arthritis and Interstitial Lung Disease 1 (TRAIL1) trial is a randomized, double-blind, placebo-controlled, phase 2 study of the safety, tolerability, and efficacy of pirfenidone in patients with RA-ILD 4.
Medications Being Investigated
- Janus kinase inhibitors (JAKi) have been shown to be effective in RA-ILD, with a pooled analysis demonstrating an increase in %pFVC and %pDLCO, as well as improvement in thoracic HRCT scans 5.
- Nintedanib, an antifibrotic drug, has been suggested to be effective in patients with progressive lung fibrosis, including RA-ILD 3.
- Pirfenidone is being investigated in the TRAIL1 trial, with the primary endpoint being the incidence of decline in percent predicted forced vital capacity or death during the 52-week study period 4.
Ongoing Research and Gaps in Knowledge
- There is a substantial gap in RA-ILD treatment, with a need for more controlled trials to evaluate the effect of RA treatments on the ILD course 3.
- International collaborative efforts are needed to address the lack of recommendations for screening high-risk patients and developing specific therapeutic strategies for RA-ILD 3.
- Further research is needed to understand the pathogenesis, diagnosis, and treatment of RA-ILD, including the role of disease-modifying anti-rheumatic drugs (DMARDs) in preventing or worsening ILD 6.