ArticleRespiratory research2025
A serum biomarker-based index for predicting progressive pulmonary fibrosis in patients with anti-synthetase syndrome: the LOCCM index.
Article in Respiratory research, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- Disparate periphery and lung immune microenvironments induced monocyte-macrophage activation as a key factor in anti-synthetase syndrome-associated interstitial lung disease.Respiratory research · 2026Article
- Advances in Biomarkers and Therapeutic Targets for Idiopathic Inflammatory Myopathies Related Lung Disease.Clinical reviews in allergy & immunology · 2026Review
- Distinct phenotypic clusters associated with progressive pulmonary fibrosis in anti-synthetase syndrome-associated interstitial lung disease.Frontiers in immunology · 2026Article
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11 authors.
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Abstract
backgroundInterstitial lung disease (ILD) is the most prominent clinical feature of antisynthetase syndrome (ASyS), with a subset of patients developing progressive pulmonary fibrosis (PPF), which is associated with a poor prognosis. However, the prevalence, clinical characteristics, and predictive factors of PPF in patients with ASyS remains unclear.
methodsThis longitudinal cohort study retrospectively enrolled 643 patients with ASyS-associated ILD. Clinical data, outcomes, and serum biomarkers were analyzed in patients with PPF. Receiver operating characteristic curves, least absolute shrinkage and selection operator, and logistic regression were used to identify key biomarkers associated with PPF.
resultsFibrotic ILD was observed in 51.2% of patients with ASyS, while PPF was observed in 27.6% of patients with ASyS and fibrotic ILD during follow-up (median, 48.9 months). Patients with PPF were more likely to be male (37.5% vs. 23.2%, p = 0.043), have a smoking history (29.7% vs. 14.3%, p = 0.012), exhibit higher rates of dysphagia (10.9% vs. 2.4%, p = 0.017), and have lower diffusing capacity for carbon monoxide (DLCO, 47.30% vs. 57.00%, p = 0.004) at baseline compared to those without PPF. No association was observed between PPF and anti-aminoacyl transfer RNA synthetase (ARS) antibody specificity (p = 0.164). Patients with PPF had significantly worse prognosis than those without PPF (log-rank test, p < 0.001). Lactate dehydrogenase (LDH), osteopontin, CA15-3, CYFRA21-1, and matrix metalloproteinase-7 (MMP-7) were identified as key biomarkers associated with PPF and incorporated into a LOCCM index (score range: 0-5). Among patients with LOCCM scores ranging from 0 to 5, the proportions of PPF were 0.0%, 4.5%, 13.3%, 35.1%, 65.0%, and 100.0%, respectively. The LOCCM index demonstrated strong predictive power for PPF in patients with ASyS and fibrotic ILD (AUC: 0.865, p < 0.001). Internal validations further confirmed its excellent discriminative performance and good calibration. Longitudinal assessment revealed that LOCCM index scores increased in patients with PPF and decreased in those without PPF.
conclusionsApproximately half of patients with ASyS-ILD presented with fibrotic ILD, among whom approximately one-quarter developed PPF. The LOCCM index is a promising tool for identifying patients with ASyS at risk for PPF development.
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