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GLASGOW, Scotland — The British Society for Rheumatology’s first guideline for the diagnosis and management of systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD) incorporates the latest data available as well as the newest treatment for progressive pulmonary fibrosis (PPF), nerandomilast, even though it is not yet approved in the UK or EU.

Guidelines on screening, diagnosis, and management of ILD in patients with connective tissue diseases already exist from the European Alliance of Associations for Rheumatology/European Respiratory Society and also the American College of Rheumatology, but coauthor Mia Rodziewicz, MBBS, a National Institute for Health and Care Research Academic Clinical Lecturer at The University of Manchester in Manchester, England, said that a guideline particular to the UK was needed that considers the structure and funding of the UK National Health Service as well as local drug licensing.

“SARD-ILD is a very heterogeneous group of conditions, which has a very variable prevalence as well as clinical course,” said Rodziewicz, who presented highlights of the guidelines at the British Society for Rheumatology (BSR) 2026 Annual Meeting.

The guideline covers screening for ILD in patients with SARDs, monitoring and management of patients with established ILD, and disease-modifying antirheumatic drug-induced pneumonitis.

It spans the gamut of SARDs associated with ILD, which according to data from a recent meta-analysis has a prevalence of about 47% among people with systemic sclerosis (SSc), 56% for mixed connective tissue disease, 41% for idiopathic inflammatory myositis (IIM),17% for primary Sjögren disease (SD), 11% for rheumatoid arthritis (RA), and 6% for systemic lupus erythematosus (SLE). The guideline also covers those with ILD associated with axial spondyloarthritis and antineutrophil cytoplasmic antibody-associated vasculitis, as well as patients with established ILD who are suspected of having a SARD.

Developing the British Guidelines

The guideline working group consisted of 28 members, including 13 rheumatologists, seven respiratory physicians, three specialist trainees, two experts by experience, one respiratory nurse specialist, one thoracic radiologist, and one specialist pharmacist.

Rodziewicz reported that for a statement to be included in the guideline, there had to be a minimum of 80% agreement among each of the working group members.

At the meeting, Rodziewicz touched briefly on a few aspects of the guidelines, which are currently under review and will likely be published in a few months.

This included talking about general screening recommendations, use of immunosuppressant therapy, and antifibrotic therapy for PPF. Overall, she said that assessment by a multidisciplinary team was important in the diagnosis, screening and management of patients with SARD-ILD. Moreover, those with ILD suspected of having a SARD who may be referred into rheumatology from other services should be seen or at least discussed with specialist ILD teams.

Screening and Monitoring for SARD-ILDs

In all patients with SSc and specific subgroups of patients with IIM, the guideline recommends screening with high-resolution CT (HRCT) as well as full pulmonary function tests (PFTs) at diagnosis, irrespective of whether the patient has symptoms or signs of respiratory disease, Rodziewicz said.

The specific IIM subgroups include people with antisynthetase-associated autoantibodies, anti-MDA5 positivity, and scleroderma overlap (eg, clinical features and autoantibodies).

Patients with RA with risk factor(s) for developing ILD — male sex, high rheumatoid factor or high anticitrullinated peptide antibody titers, history of smoking, high BMI, or age older than 60 years at disease onset or high disease activity at onset should undergo HRCT and PFT regardless of their respiratory history.

For all other SARDs, a thorough respiratory history and examination should be performed, but only those with positive findings should then undergo HRCT and PFT.

“Importantly, the group recommended against the use of chest x-ray as a screening tool for interstitial lung disease due to its incredibly low sensitivity,” Rodziewicz said.

To monitor patients, the group generally recommends PFTs every 3-6 months initially, then at 6- to 12-month intervals once stable, and repeating HRCT if there are new or worsening respiratory symptoms. The exception is for people with SSc-ILD, who should undergo PFTs every 3-6 months for at least 3-5 years.

Treating SARD-ILDs

General advice for inflammatory-related ILDs is to use glucocorticoids with early introduction of immunosuppressive treatment as the initial management strategy, which is supported by strong evidence, Rodziewicz said.

For first-line immunosuppressive treatment, mycophenolate mofetil (MMF) should be the ‘go-to’ option, with azathioprine and calcineurin inhibitors (CNIs) considered alternatives if MMF is not tolerated or is contraindicated. However, CNIs should be avoided in those with SSc “due to the association with renal crisis,” she said.

The guideline advises escalating immunosuppressant dosing in cases where there is evidence of disease progression despite optimal dosing, contraindications, or failure to tolerate first-line treatment.

Second-line options include intravenous infusion of rituximab, cyclophosphamide, or both, except for cases of significant or rapidly progressive ILD in which these drugs might be considered first-line options.

Don’t Delay If ILD Rapidly Progresses

“Please don’t delay treatment in rapidly progressing interstitial lung disease,” Rodziewicz urged. This can happen with any patient with SARD-ILD and “these patients require early and aggressive immune suppression.”

Rapidly progressive-ILD involves worsening dyspnea, hypoxia, or radiologic signs within 3 months of symptom onset in the absence of alternative etiology. Urgent multidisciplinary team discussion is required with initiation of intravenous glucocorticoids and cyclophosphamide, with or without rituximab.

For IIM-ILD that is rapidly progressing, immunosuppressant combinations (such as cyclophosphamide and a calcineurin inhibitor) could be considered, possibly adding treatment with tofacitinib, intravenous immunoglobulin, or plasma exchange. Discussion with an extracorporeal membrane oxygenation center should be considered, Rodziewicz said.

PPF

PPF is likely if the following signs occurred within a 2-year period: the percentage predicted forced vital capacity declined by 10% or more, it declines by 5%-10% with worsening of fibrosis on HRCT or there is worsening respiratory symptoms, or if there are worsening respiratory symptoms and worsening of fibrosis on HRCT.

The guidance recommends nintedanib or nerandomilast in combination with immunosuppressive therapy or these two antifibrotic drugs can be used in combination without immunosuppressants where appropriate. If tolerance is poor, dose reduction may be needed.

“Our guidelines are somewhat aspirational,” said Rodziewicz, referring to the inclusion of nerandomilast which is still under review by the National Institute for Health and Care Excellence, “but we’re hoping, based on the evidence, that this drug will be commissioned for use in this patient population.”

A key takeaway from Rodziewicz was to seek help both locally and within the multidisciplinary team and remember that were national ILD specialist services available that could be referred into.

Rodziewicz reported receiving research funding from the Medical Research Council, Roche Pharma, Eli Lilly, UCB Pharma, Novartis, the University of Liverpool, and the University of Manchester (paid to employer).

Sara Freeman is a medical journalist based in London, England.