Interstitial lung disease (ILD) is a common and potentially fatal complication of systemic autoimmune rheumatic diseases (SARDs), such as rheumatoid arthritis (RA) and systemic sclerosis (SSc). ILD may be the first sign of disease or develop later, often with variable clinical courses. Early detection is critical, as delayed diagnosis contributes to poor outcomes. In response, the 2023 American College of Rheumatology (ACR) guidelines emphasize routine screening and risk-based strategies to improve identification and management of SARD-associated ILD (SARD-ILD).¹

“Screening for SARD-ILD & BSR guidelines” was presented at the British Society for Rheumatology Annual Conference 2025, Manchester, UK; 28-30 April.

Q. What is the current best evidence regarding screening for ILD in patients with systemic autoimmune rheumatic diseases (SARDs)?

The best current evidence supports routine screening for ILD in patients with SARDs who are considered at risk. According to the 2023 guidelines from the ACR, patients with certain SARDs should undergo early and systematic evaluation for ILD.¹ This recommendation is underpinned by data indicating that ILD is both a common and potentially fatal complication of systemic autoimmune diseases, often contributing to early mortality. Moreover, ILD may be the first clinical sign of a SARD, and SARD-ILD can present with diverse clinical features and disease trajectories. Studies have shown that patients with SARD-ILD face a significantly elevated risk of death within five years, with reported mortality rates up to 39%.² Therefore, early detection through screening is essential to improving outcomes.

Q. Which SARDs carry the highest risk for ILD, and how should that influence screening strategies?

Among the systemic autoimmune rheumatic diseases, RA and SSc are most commonly associated with ILD. Epidemiological data from large cohort studies suggest that RA accounts for approximately 39% and SSc for 31% of SARD-ILD.³ Other diseases, such as polymyositis, systemic lupus erythematosus (SLE), and Sjögren’s syndrome, also carry risk, but to a lesser extent. Screening strategies should prioritize patients with known risk factors, such as older age, male sex, smoking history, and the presence of specific autoantibodies.^4,5,6 Early screening in these populations is essential, as ILD may develop early in the disease course or even present before the rheumatic disease is fully diagnosed.⁷

Q. What screening tools or modalities are most effective and practical in early ILD detection?

The most effective and practical modalities for early ILD detection in patients with SARDs are a combination of pulmonary function tests (PFTs), high-resolution computed tomography (HRCT), and autoimmune serological testing.^8,9 PFTs, particularly measurements of forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLCO), provide valuable baseline and longitudinal information. A decline in FVC, especially one exceeding 10%, is an indicator of disease progression.⁸ The ACR 2023 guidelines recommend, for screening individuals with SARDs at risk for ILD, a conditional use of PFTs and HRCT of the chest. The guidelines also conditionally recommend against using the 6-minute walk test (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy for screening purposes, and strongly recommend against screening with surgical lung biopsy.¹ Additionally, a full autoimmune serological panel can help confirm the presence of a systemic autoimmune condition and support the diagnosis of ILD when pulmonary symptoms are non-specific or absent.⁶

Q. Based on current evidence, what changes would you recommend to current clinical practice or guidelines?

In light of current evidence, several modifications to clinical practice and guidelines are warranted to improve the detection and management of SARD-ILD. There should be a routine implementation of PFTs and HRCT imaging for patients diagnosed with high-risk SARDs within the first few years of disease onset. Assessment should incorporate demographic, clinical, and serological factors, which guide the frequency and intensity of screening. Monitoring using repeat PFTs and imaging should be standardized to enable early identification of progressive pulmonary fibrosis, which may occur in up to 40% of patients with fibrosing SARD-ILDs.¹⁰ Additionally, lung ultrasound should be integrated into rheumatology practice as a supplemental tool for ILD surveillance, particularly in resource-limited environments. Finally, a multidisciplinary team approach is essential to ensure accurate diagnosis, timely intervention, and optimal management of these complex conditions.¹¹

References

1. 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 & Rheumatology. 2024;76:1201–1213. Doi: 10.1002/art.42860
2. Hyldgaard C, Hilberg O, Pedersen AB, et al. A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis. 2017;76:1700–1706. Doi: 10.1136/annrheumdis-2017-211138.
3. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968. Doi: 10.1056/NEJMra2005230.
4. Kim H, Cho SK, Song YJ, et al. Clinical characteristics of rheumatoid arthritis patients with interstitial lung disease: baseline data of a single-center prospective cohort. Arthritis Res Ther. 2023;25:43. Doi: 10.1186/s13075-023-03024-8.
5. Esposito AJ, Chu SG, Madan R, et al. Thoracic Manifestations of Rheumatoid Arthritis. Clin Chest Med. 2019;40:545–560.
6. Kuwana M, Gil-Vila A, Selva-O’Callaghan A. Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders. Ther Adv Musculoskelet Dis. 2021;27:13:1759720X211032457. Doi: 10.1177/1759720X211032457.
7. Mathai SC, Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016;352:h6819. Doi: 10.1136/bmj.h6819.
8. Cottin V, Hirani NA, Hotchkin DV, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180076. DOI: 10.1183/16000617.0076-2018.
9. Kalchiem-Dekel O, Galvin JR, Burke AP, et al. Interstitial Lung Disease and Pulmonary Fibrosis: A Practical Approach for General Medicine Physicians with Focus on the Medical History. J Clin Med. 2018;7:476. Doi: 10.3390/jcm7120476.
10. Olson A, Hartmann N, Patnaik P, et al. Estimation of the Prevalence of Progressive Fibrosing Interstitial Lung Diseases: Systematic Literature Review and Data from a Physician Survey. Adv Ther. 2021;38(2):854–867. Doi: 10.1007/s12325-020-01578-6.
11. George PM, Spagnolo P, Kreuter M, et al; Erice ILD working group. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med. 2020;8:925–934. Doi: 10.1016/S2213-2600(20)30355-6.

Further content in respiratory disorders and rheumatic diseases.

Editor: Victoria Jones, Senior Content Editor.

Disclosures: This short article was prepared by touchIMMUNOLOGY in collaboration with Muhammad Nisar. touchIMMUNOLOGY utilize AI as an editorial tool (ChatGPT (GPT-4o) [Large language model]. https://chat.openai.com/chat.) The content was developed and edited by human editors. No fees or funding were associated with its publication.

Muhammad Nisar discloses consulting for, receiving grant/research support and honoraria from, serving on advisory boards and speaker’s bureaus for: Boeringher Ingleheim.

Cite: Muhammad Nisar. Screening for Interstitial Lung Disease in Systemic Autoimmune Rheumatic Diseases: Muhammad Nisar, BSR 2025. touchIMMUNOLOGY. June 2025.

Muhammad Nisar is a Consultant Rheumatologist and Physician at Bedfordshire Hospitals NHSFT, having completed his dual speciality training in the East of England. He is also the Director of Research and Development at the organisation. Nationally, he is an elected clinical entrepreneur, Deputy Chair of BSR Heberden’s Committee and Chair of SARD-ILD BSR Guidelines Group. He is the lead clinician for award winning early arthritis service, metabolic bone health delivery, research on advanced therapies and inflammatory arthritis associated lung diseases MDT service. Dr Nisar has been involved in more than 80 clinical trials utilising advanced therapies for autoimmune and skeletal conditions. He has won several awards from prestigious bodies including the NHS, RCP and BSR. His other main interests include early inflammatory arthritis including ultrasound use and crystal arthritides.

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