The guidelines include disease-specific recommendations on screening, monitoring disease progression, and treatment options for ILD.
The guidelines are a collaboration between EULAR and the Europe an Respiratory Society to provide comprehensive, disease-specific recommendations for the screening, diagnosis, and management of ILD in patients with connective tissue disease s , including SSc, MCTD, "These are the first recommendations 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 is that it is interdisciplinary work," he said.The guidelines strongly recommend systematic screening for ILD with high-resolution computed tomography in all patients with SSc and MCTD, regardless of risk factors. This recommendation also applies to patients with IIM, except those with 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 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, 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.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.The new treatment recommendations"not only list which treatment options are available for every disease but also give hints 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 withOther 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 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."Hellmich has reported financial relationships with AbbVie, Amgen, AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, Chugai, GSK, InflaRx, Janssen, MSD, Novartis, Pfizer, Phadia, Roche, and CSL Vifor. Hoffmann-Vold has reported financial relationships with Boehringer Ingelheim, Janssen, Medscape, MSD, Novartis, Roche, AbbVie, Arxx Therapeutics, Bristol Myers Squibb, Genentech, Pliant Therapeutics, and Werfen. All material on this website is protected by copyright, Copyright © 1994-2025 by WebMD LLC. This website also contains material copyrighted by 3rd parties.
Rheumatoid Arthritis (RA) Clinical Guidelines Guidelines Preventive Screening Screening Europe European Lung Tissue Connective Tissue Disease Mixed Connective Tissue Disease Mixed Connective-Tissue Disease Sharp Syndrome Interstitial Lung Disease Biologic Therapy Biologics Computed Tomography CT Ct Scan Inclusion Body Myositis
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