Arthritis & rheumatology (Hoboken, N.J.) 2024 Jul 08
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.
Abstract
Objective
We provide evidence-based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease.
Methods
We developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation.
Results
Fifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high-resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6-minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs.
Conclusion
This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.
Related Questions
What findings on routine monitoring PFTs prompt you to pursue HRCT in your patients with SARDs?
That’s an excellent question, and the strategy might vary somewhat by the specific SARD, but in general, in any SARD patient undergoing annual PFTs, the presence of any of these should prompt an HRCT to evaluate for the development of ILD. FVC drop ≥ 10% DLCO drop ≥ 15% Moderate decline in FVC (5-9...
What minimum clinical features or risk factors drive you to obtain a baseline HRCT in a patient with RA or Sjogren's disease?
Overall, about 10% of RA patients will develop clinically significant ILD, as will 20% of those with SjD. Those are a significant number of people, and ILD should be on our radar as clinicians. Knowledge of risk factors for ILD can help us focus on people most likely to be affected. Several risk fac...
How do you approach screening for ILD in patients with a diagnosis of MCTD given the recommendation discrepancies between the most recent EULAR and ACR/CHEST guidelines?
Another excellent question! While the EULAR guidelines treat MCTD as SSc-equivalent and suggest universal screening, ACR/CHEST guidelines suggest risk-stratified screening with emphasis on symptoms, PFT abnormalities, and high-risk phenotypes.Prevalence of ILD in MCTD can be high, in the range of 30...
Do you recommend maintaining the same monitoring interval of PFTs every 3–6 months with HRCT as indicated for patients with anti-MDA5 dermatomyositis, or do you recommend closer surveillance in this group?
Closer surveillance may be needed at diagnosis of ILD in anti-MDA5 DM at every 3 months for 1st year. But typically, in my experience, patients' symptoms progress faster than every 3 months, so rapidly progressive ILD is diagnosed clinically.
How often are you repeating screening PFTs in patients with SARDs who have 3 or more years of normal or stable PFTs?
The answer to this question is complex and needs to be tailored to the individual patient’s risk for ILD and the particular SARD.Approximately 30-40% of patients with systemic sclerosis (SSc) will develop ILD, typically within the first 5 years after the first non-Raynaud’s manifestation and rarely ...
Do you screen for interstitial lung disease in patients with newly diagnosed polymyositis or dermatomyositis in the absence of respiratory symptoms?
I do screen all newly diagnosed IIM patients with PFTs and chest CT. This has a double purpose: establishing a baseline of lung function and, screening for lung cancer. While the patient might not have lung symptoms on presentation, respiratory involvement can manifest later on the course of the d...
How do you screen rheumatoid arthritis patients for lung disease (modality, frequency, patient selection)?
Theoretically, we are supposed to screen these patients obtaining a baseline chest X-ray before starting DMARD therapy. As I remember, these guidelines were formulated when MTX was still blamed for MTX lung disease although presently, even the presence of underlying ILD is not necessarily a contrain...