AMERICAN COLLEGE of RHEUMATOLOGY Screening and Monitoring of Interstitial Lung Disease Instructions

Screening and Monitoring of Interstitial Lung Disease
Instructions

Screening and Monitoring of Interstitial Lung Disease

2023 American College of Rheumatology (ACR) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune
Rheumatic Disease
Guideline Summary
This guideline was developed to provide recommendations for the screening of Interstitial Lung Disease (ILD) in people with Systemic Autoimmune Rheumatic Diseases (SARDs) [Rheumatoid Arthritis (RA), Systemic Sclerosis (SSc), Idiopathic Inflammatory Myositis (IIM including polymyositis, dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy), Mixed Connective Tissue Disease (MCTD), and Sjögren’s Disease (SjD)] associated with the greatest risk of ILD, and for monitoring for ILD progression. These recommendations facilitate rheumatologists’ identification of ILD among people with SARDs and will assist in optimizing the co-management of people with SARDs-associated ILD by rheumatologists and pulmonologists.
Table 1. Summary of recommendations for screening of SARD-ILD
Summary of recommendations
For people with SARDs at increased risk of developing ILD, we conditionally recommend screening with PFTs.
For people with SARDs at increased risk of developing ILD, we conditionally recommend screening with HRCT of the chest.
For people with SARDs at increased risk of developing ILD, we conditionally recommend screening with HRCT chest and PFTs over PFTs alone.
For people with SARDs at increased risk of developing ILD, we conditionally recommend against screening with 6MWD.
For people with SARDs at increased risk of developing ILD, we conditionally recommend against screening with chest radiography.
For people with SARDs at increased risk of developing ILD, we conditionally recommend against screening with ambulatory desaturation testing.
For people with SARDs at increased risk of developing ILD, we conditionally recommend against screening with bronchoscopy.
For people with SARDs at increased risk of developing ILD, we strongly recommend against screening with surgical lung biopsy.
Table 2. Summary of Recommendations for Monitoring for ILD Progression
Summary of recommendations
For people with SARDs-ILD, we conditionally recommend monitoring with PFTs.
For people with SARDs-ILD, we conditionally recommend monitoring with HRCT chest.
For people with SARDs-ILD, we conditionally recommend monitoring with PFTs and HRCT chest over PFTs alone.
For people with SARDs-ILD, we conditionally recommend monitoring with ambulatory desaturation testing.
For people with SARDs-ILD, we conditionally recommend against monitoring with chest radiography.
For people with SARDs-ILD, we conditionally recommend against monitoring with 6MWD.
For people with SARDs-ILD, we conditionally recommend against monitoring with bronchoscopy.
For people with IIM-ILD and SSc-ILD, we suggest PFTs for monitoring every 3-6 months rather than either shorter or longer intervals, for the first year, then less frequently once stable.
For people with RA-ILD, SjD-ILD, and MCTD-ILD, we suggest PFTs for monitoring every 3-12 months rather than shorter or longer intervals, for the first year, then less frequently once stable.
For people with SARDs-ILD, we do not provide guidance about frequency of routine HRCT chest for monitoring ILD but suggest HRCT when clinically indicated.
For people with SARDs-ILD, we suggest assessment for ambulatory desaturation every 3-12 months rather than at shorter or longer intervals.

Figure 1; Recommendations for ILD Screening and Monitoring

• See Table 1 for risk factors for interstitial lung disease
• Ambulatory desaturation can be done during a routine office visit or as part of 6-minute walk testing SARD = systemic autoimmune rheumatic disease; ILD = interstitial lung disease; CT = computed tomography; liM = idiopathic inflammatory myopathy; SSc = systemic sclerosis; RA = rheumatoid arthritis; SjS = Sj6gren’s disease, MCTD = mixed connective tissue disease
  Note. Frequency of monitoring in italics are suggestions to assist application of the recommendations. Figure 2: Interstitial lung disease screening and monitoring tests recommended against. Tests shown are recommended against for routine use, although examples are provided when these tests may have utility for assessing patients or ruling out other conditions.
  PFT = pulmonary function test
  This summary was approved by the ACR Board of Directors on 12 August 2023. These recommendations are included in a full manuscript, pending peer review, which was submitted for publication in Arthritis & Rheumatology and Arthritis Care and Research.

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