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Medscape
a day ago
- Health
- Medscape
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 .
Medscape
12-05-2025
- Health
- Medscape
New Model Predicts Risk for Progression of RA-ILD
The risk for progression of rheumatoid arthritis–associated interstitial lung disease (RA-ILD) was higher in patients with a usual interstitial pneumonia (UIP) pattern, an ILD extent of > 10%, and a lower diffusing capacity for carbon monoxide (DLCO), along with elevated levels of certain biomarkers. A prediction modelbased on these clinical factors and biomarkers effectively stratified patients by their risk for disease progression. METHODOLOGY: Researchers analyzed data from a prospective cohort study to identify clinical risk factors for the progression of RA-ILD, as defined by the recent progressive pulmonary fibrosis (PPF) criteria, and to develop a corresponding prediction model. They enrolled 138 adult patients diagnosed with RA-ILD (mean age, 66.4 years; 30.4% men) between January 2015 and July 2018. Patients were followed up annually for 3 years to determine disease progression, defined as meeting the PPF criteria for both pulmonary physiology and radiographical worsening domains, assessed using pulmonary function tests and CT scans, respectively. RA disease activity was assessed, and information on prescription medications and demographics was collected at each visit. Levels of autoantibodies (anti–cyclic citrullinated peptide [anti-CCP] and anti–citrullinated enolase peptide) , pulmonary damage biomarkers ( Krebs von den Lungen 6 [KL-6], matrix metallopeptidase 7, and human surfactant protein D [hSP-D]), and certain inflammatory markers were evaluated. TAKEAWAY: Over a median follow-up period of 2.9 years, the progression of RA-ILD occurred in 35% of patients; a higher risk for progression was noted in patients with low and moderate disease activity scores than in those in remission. An increased risk for progression was noted in patients with a definitive or probable UIP pattern (adjusted hazard ratio [aHR], 2.72; P = .004) and an ILD extent of > 10% (aHR, 3.51; P < .001). Further, a lower DLCO was associated with a higher risk for progression (aHR, 0.67 per SD; P = .01). = .004) and an ILD extent of > 10% (aHR, 3.51; < .001). Further, a lower DLCO was associated with a higher risk for progression (aHR, 0.67 per SD; = .01). The key biomarkers predicting a higher risk for progression were higher levels of anti-CCP (aHR, 1.33 per unit), KL-6 (aHR, 1.41 per SD), and hSP-D (aHR, 1.51 per SD; P < .05 for all). < .05 for all). The prediction model incorporating clinical factors and KL-6 level achieved an area under the curve of 0.75, with the high-risk group showing a positive predictive value of 85.7% and the low-risk group showing a negative prediction value of 94.7% for progression. IN PRACTICE: 'The implementation of this model may facilitate the timely initiation of appropriate treatment by shortening the time required to fulfill the PPF criteria,' the authors wrote. SOURCE: This study was led by Sung Hae Chang, Soonchunhyang University College of Medicine, Cheonan, Republic of Korea. It was published online on April 17, 2025, in Seminars in Arthritis & Rheumatism . LIMITATIONS: This study cohort primarily consisted of patients with mild ILD and was limited to a Korean population, potentially limiting generalizability. Factors such as pulmonary symptoms and oxygen saturation were not evaluated. The sample size was relatively small, and this study lacked an external validation cohort. DISCLOSURES: Some authors were supported by various sources, including the National Research Foundation of Korea, Rheumatology Research Foundation Scientist Development Award, National Institute of Arthritis and Musculoskeletal and Skin Diseases, and several others. One author received support from the Soonchunhyang University Research Fund. Another author reported receiving support from and performing consultancy for several pharmaceutical companies.
