Interstitial lung disease (ILD) remains a critical concern in the realm of rheumatic diseases, which significantly impact patient outcomes and quality of life. As our understanding of this complex interplay evolves, so too must our approaches to risk assessment, screening, diagnosis, and monitoring. That’s why we’re taking an in-depth look at current best practices and potential future directions.
Risk Factors
Systemic sclerosis, rheumatoid arthritis, and idiopathic inflammatory myopathies stand out as particularly high-risk conditions for ILD. However, the risk extends to other autoimmune rheumatic diseases, including Sjögren's syndrome and mixed connective tissue disease.
In addition to these conditions, recent research has shed light on the following risk factors:
Screening and Monitoring Strategies
Our approach to screening for ILD in rheumatic diseases is rapidly evolving. In fact, the 2023 American College of Rheumatology guidelines mark a significant milestone as they provide the first evidence-based recommendations for ILD screening and monitoring in systemic autoimmune rheumatic diseases.
According to the guidelines, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) of the chest are conditionally recommended for screening high-risk individuals. They advise against routine use of the 6-minute walk test, chest radiography, and bronchoscopy. Notably, this challenges some traditional practices and underscores the need for evidence-based approaches.
Additionally, the guidelines do not recommend utilizing surgical lung biopsy for screening purposes. This position aligns with the growing emphasis on non-invasive diagnostic techniques and the recognition of the potential risks associated with invasive procedures.
Once ILD is diagnosed, the ACR guidelines conditionally recommend using PFTs, HRCT, and ambulatory desaturation testing to monitor patients, but the frequency of these assessments should be individualized based on disease severity and progression rate.
Diagnosing and monitoring ILD in rheumatic diseases requires a nuanced, multidisciplinary approach. In addition to utilizing the latest clinical guidelines, proactive collaboration between pulmonologists and rheumatologists has shown promise in increasing diagnostic accuracy, reducing unnecessary invasive procedures, and optimizing comprehensive patient care.
Future Directions
Recent research has highlighted the potential of novel screening tools. Transthoracic ultrasound, for instance, has emerged as a promising technique for evaluating ILD, particularly in systemic sclerosis and rheumatoid arthritis. While more research is needed, this method could offer a radiation-free alternative for frequent monitoring, addressing concerns about cumulative radiation exposure from repeated HRCT scans.
The role of biomarkers in ILD screening and monitoring is another frontier being explored. Markers such as KL-6 and CCL2 show promise for both detection and prognostic assessment of ILD. However, their integration into clinical practice remains a work in progress, highlighting the need for further research and standardization.
Other emerging technologies that may enhance our monitoring capabilities include MRI and PET/CT scans. MRI scans could potentially assess both morphological and functional aspects of lung involvement, while PET/CT scans could provide insights into disease activity and metabolic changes.
These are all potential avenues that require additional research, but by embracing evidence-based practices, fostering multidisciplinary collaboration, and remaining open to innovative approaches, we can work to mitigate the impact of ILD on patients with rheumatic diseases.
References:
ACR introduces new guidelines to screen, monitor and treat interstitial lung disease in patients with rheumatic conditions. American College of Rheumatology. August 22, 2023. Accessed January 14, 2025. https://rheumatology.org/press-releases/acr-introduces-new-guidelines-to-screen-monitor-and-treat-interstitial-lung-disease-in-patients-with-rheumatic-conditions.
Bosello SL, Beretta L, Del Papa N, et al. Interstitial lung disease associated with autoimmune rheumatic diseases: Checklists for clinical practice. National Library of Medicine. October 15, 2021. Accessed January 14, 2025. https://pmc.ncbi.nlm.nih.gov/articles/PMC8554062/.
Hackner K, Hütter L, Flick H, et al. Screening for rheumatoid arthritis-associated interstitial lung disease-a delphi-based consensus statement. PubMed Central. March 2024. Accessed January 14, 2025. https://pmc.ncbi.nlm.nih.gov/articles/PMC10902070/.
Hannah J, Rodziewicz M, Mehta P, et al. Diagnosis and management of systemic autoimmune rheumatic disease-related interstitial lung disease: British Society for Rheumatology Guideline Scope | Rheumatology advances in Practice | Oxford academic. Rheumatology Advances in Practice. April 18, 2024. Accessed January 14, 2025. https://academic.oup.com/rheumap/article/8/2/rkae056/7651208.
Hoffmann T, Oelzner P, Teichgräber U, et al. Diagnosing lung involvement in inflammatory rheumatic diseases-where do we currently stand? Frontiers in Medicine. December 19, 2022. Accessed January 14, 2025. https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2022.1101448/full.
How to monitor and manage progressive ild. PulmonaryFibrosis360.com. February 27, 2024. Accessed January 14, 2025. https://www.pulmonaryfibrosis360.com/rheums/ra-ild/monitoring-and-management-of-ild-progression-in-ra-ild.
Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (acr)/american college of chest physicians (chest) guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic diseases. PubMed. July 8, 2024. Accessed January 14, 2025. https://pubmed.ncbi.nlm.nih.gov/38973714/.
Morais A, Duarte AC, Fernandes MO, et al. Early detection of interstitial lung disease in rheumatic diseases: A joint statement from the Portuguese Pulmonology Society, the Portuguese Rheumatology Society, and the Portuguese Radiology and Nuclear Medicine Society. Pulmonology Journal. December 26, 2023. Accessed January 14, 2025. https://journalpulmonology.org/en-early-detection-interstitial-lung-disease-avance-S2531043723002052.
Rheumatoid arthritis and lung disease: Arthritis foundation. Arthritis Foundation. Accessed January 14, 2025. https://www.arthritis.org/diseases/more-about/what-you-need-to-know-about-ra-and-lung-disease.
Sebastiani M, Vacchi C, Cassone G, Manfredi A. Diagnosis, clinical features and management of interstitial lung diseases in rheumatic disorders: Still A long journey. PubMed Central. January 14, 2022. Accessed January 14, 2025. https://pmc.ncbi.nlm.nih.gov/articles/PMC8781573/.
Interstitial lung disease (ILD) in rheumatic conditions can significantly impact patient outcomes and quality of life. New research has shed light on risk factors as well as screening and monitoring strategies, improving our understanding of how we can mitigate the impact of ILD on these patients. Read about recent advancements and recommendations for comprehensive management.