Screening and Monitoring Chapter 2: Screening Strategies by Disease

Watch Chapter 3 here.

Hello, and welcome back to the ILD Explainer Video Series. I am Dr Elana Bernstein, an associate professor of medicine at Columbia University Vagelos College of Physicians & Surgeons and an associate attending at New York-Presbyterian Hospital. I am also the founder and director of the Columbia University/New York-Presbyterian Scleroderma Program. 

This series focuses on screening and monitoring ILDs. In this video, we’ll simplify the 2023 American College of Rheumatology—known as ACR—and the American College of Chest Physicians—known as CHEST— recommendations into practical clinic tools, outline disease-specific screening intervals and monitoring thresholds, demonstrate what progression looks like on tests and imaging, and share workflow strategies to embed these guidelines into daily practice.  

Let’s start with screening: who should we screen, and how often? 

This guideline organizes screening recommendations by the following SARDs and their risk factor categories: systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), mixed connective tissue disease (MCTD), and Sjögren’s disease (SjD).

For people with SARDs, the first step is to determine whether they are at an increased risk for ILD.

Suppose a patient is considered high risk for an ILD. In that case, the recommended assessment includes pulmonary function testing (PFT), which consists of spirometry, lung volumes (like forced vital capacity [FVC]), and diffusion capacity (like diffusing capacity of the lung for carbon monoxide [DLCO]), along with a high-resolution computed tomography (HRCT) scan of the chest.

Let’s return to our patient, Maria. With current pulmonary symptoms raising suspicion of ILD, Maria underwent a diagnostic workup including PFT and HRCT, and her care team confirmed that she has SARD-ILD.  

Once a patient receives a diagnosis, the next step is ongoing monitoring. The guidelines suggest repeating PFTs every 3 to 12 months during the first year, then less frequently once the disease is stable for RA. Health care professionals should also perform ambulatory desaturation testing every 3 to 12 months and repeat HRCT scans as needed to assess disease progression. 

One of the most common questions we hear is: “How often should I monitor patients who are diagnosed with SARD-ILD?”  

The guidelines provide some suggestions:  

• Patients with a SARD who are at high risk for developing ILD or who show signs and symptoms suggestive of ILD should undergo baseline testing with PFTs and HRCT.  

• From there, PFTs should be repeated more frequently in higher-risk diseases—every 3 to 6 months during the first year for SSc or IIMs. For RA, Sjögren's disease, and mixed connective tissue disease, PFTs may be repeated every 3 to 12 months in the first year, and then less often once the disease is stable. 

• Ambulatory desaturation testing, which measures oxygen saturation during walking or light activity to identify exertional desaturation and determine oxygen needs, should be performed every 3 to 12 months. 

• Repeat HRCT when symptoms change, PFTs worsen, or there is concern for progression.

Maria’s case illustrates how structured assessment and regular monitoring can change the disease trajectory, allowing us to intervene earlier and preserve lung function.  

The final step is to embed these recommendations into daily clinical workflow, whether through EMR prompts, standardized order sets, or routine checklists, so that screening, monitoring, and defining progression become second nature in practice. This way, we can translate guidelines into better outcomes for patients. 

For Maria and your patients, these steps can make all the difference. 

Guidelines are not obstacles—they are tools. Use them confidently, and you can change the course of ILD for your patients. 

6MWD – 6-minute walk distance 
ACR – American College of Rheumatology 
Architectural distortion – Irreversible displacement of normal lung structures due to fibrosis 
CHEST – American College of Chest Physicians 
CTD-ILD – Connective tissue disease–associated interstitial lung disease 
DLCO – Diffusing capacity of the lung for carbon monoxide 
EMR – Electronic medical record 
FVC – Forced vital capacity 
GGO – Ground-glass opacity 
HRCT – High-resolution computed tomography 
IIM – Idiopathic inflammatory myopathy 
ILD – Interstitial lung disease 
MCTD – Mixed connective tissue disease 
MDD – Multidisciplinary discussion 
NSIP – Nonspecific interstitial pneumonia 
OP – Organizing pneumonia 
PFT – Pulmonary function testing 
PPF – Progressive pulmonary fibrosis 
Progression (ILD) – Worsening physiologic, radiologic, or symptomatic disease over time 
RA – Rheumatoid arthritis 
Radiologic progression – Worsening fibrotic features on HRCT 
Reticulation – Network of fine linear opacities caused by septal thickening 
SARD – Systemic autoimmune rheumatic disease 
SjD – Sjögren disease 
SSc – Systemic sclerosis 
Traction bronchiectasis – Irreversible airway dilation due to fibrotic pulling 
UIP – Usual interstitial pneumonia 

Antoniou KM, Distler O, Gheorghiu AM, et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG).  Eur Respir J. Published online September 11, 2025. doi:10.1183/13993003.02533-2024 

Guiot J, Miedema J, Cordeiro A, et al. Practical guidance for the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease.  Autoimmun Rev. 2024;23(6):103582. doi:10.1016/j.autrev.2024.103582 

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.  Arthritis Rheumatol. 2024;76(8):1201-1213. doi:10.1002/art.42860 

Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases.  Arthritis Care Res (Hoboken). 2024;76(8):1051-1069. doi:10.1002/acr.25348 

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Ponce MC, Sankari A, Sharma S. Pulmonary function tests. StatPearls [Internet]. StatPearls Publishing; 2025. Accessed November 13, 2025. https://www.ncbi.nlm.nih.gov/books/NBK482339/ 

Wong AW, Ryerson CJ, Guler SA. Progression of fibrosing interstitial lung disease. Respir Res. 2020;21(1):32. doi:10.1186/s12931-020-1296-3