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EULAR, ERS Issue First Guideline for Managing CTD-ILD
BARCELONA, Spain — All patients with systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) should be screened for interstitial lung disease (ILD), according to new guidelines presented at the European Alliance of Associations for Rheumatology (EULAR) 2025 Annual Meeting.
The guidelines are a collaboration between EULAR and the European Respiratory Society (ERS) to provide comprehensive, disease-specific recommendations for the screening, diagnosis, and management of ILD in patients with connective tissue diseases (CTDs), including SSc, MCTD, rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), and Sjögren's disease (SjD).
"These are the first recommendations [for ILD] that have been developed by rheumatologists and pulmonologists together in Europe," said Bernhard Hellmich, MD, of the University of Tübingen, Germany. He was not involved with the work but moderated the session during which the recommendations were presented. The ERS has published guidelines solely focusing on therapies for ILD, but "the meaningful impact [of these new guidelines] is that it is interdisciplinary work," he said.
Screening for ILD
The guidelines strongly recommend systematic screening for ILD with high-resolution computed tomography (HRCT) in all patients with SSc and MCTD, regardless of risk factors. This recommendation also applies to patients with IIM, except those with inclusion body myositis.
Patients with RA, SjD, and other CTDs should first be assessed for disease-specific risk factors. Patients with identified risk factors should be screened with HRCT.
Although some previous recommendations supported screening in SSc, "now the big news is that we should also screen patients with RA who have risk factors," Hellmich said. "We have not done this before, so this will certainly identify more patients who are eligible for therapy and may improve [outcomes] down the line."
The guidelines do not include any recommendations on screening frequency because of a lack of evidence, explained Anna-Maria Hoffmann-Vold, MD, PhD, of Oslo University Hospital, Norway, who presented the guidelines.
She advised that providers use the same approach as that used at the timepoint of diagnosis and assess for any new risk factors for ILD progression. Any patient with additional red flags such as sustained joint disease in RA should be re-screened, she said. "Of course, in any case of suspicion, re-screen the patient," she continued.
The guidelines strongly recommend against replacing HRCT with pulmonary function tests or lung ultrasound for screening for ILD; however, forced vital capacity and diffusing capacity of the lungs for carbon monoxide can be included in case of symptoms or CT abnormalities.
Monitoring After Diagnosis
The guidelines do have time recommendations for monitoring ILD progression after diagnosis. The recommendations are disease-specific and stratified by risk of ILD progression.
Clinicians can assess risk for ILD progression in the following year by looking at prior lung function tests, HRCT results, the 6-minute walking test, and risk factors for poor outcomes in ILD, which include disease-specific circulating markers, higher disease activity, and HRCT pattern and extent.
Based on these factors, clinicians can categorize patients as higher or lower risk, Hoffmann-Vold said.
"Next, you look at the disease duration of the patient — whether you have a shorter disease duration or longer disease duration in the high-risk group and the low-risk group," she explained during her presentation. "Based on this, you apply the monitoring tools we have identified."
Although some time recommendations vary by disease, for high-risk patients with any CTD, lung function tests should be performed every 3-6 months in the first years of disease and every 6-12 months thereafter. For lower-risk patients, perform lung tests every 6-12 months in the first years of disease and annually thereafter.
Treatment Recommendations
The new treatment recommendations "not only list which treatment options are available for every disease but also give hints [about] which patient might be the right one for a certain medication," Hoffmann-Vold said.
The group strongly recommends that patients with SSc-ILD with early diffuse SSc and signs of inflammation should be treated with tocilizumab and that patients with any IIM-ILD should be treated with immunosuppressive treatment, which can include glucocorticoids, calcineurin inhibitors, rituximab, mycophenolate, or azathioprine.
Other recommendations are conditional, largely because of limited patient populations and a small number of randomized controlled trials. SSc-ILD, RA-ILD, and IIM-ILD all have individual treatment algorithms.
This comprehensive guideline will "certainly help to get patients earlier into treatment," Hellmich added.
"Some of these therapies [currently] are more or less off-label," he said. But if they are included in EULAR guidelines, "people are more likely to use them in clinical practice and to improve prognosis."
The full guidelines will be published next month in Annals of the Rheumatic Diseases and European Respiratory Journal .
