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Journal Article Ppublish 13 citations

PubMed: 36929316 DOI: 10.1007/s10067-023-06570-3

Prediction of progressive pulmonary fibrosis in patients with anti-synthetase syndrome-associated interstitial lung disease.

Clin Rheumatol 42(7):1917-1929

Authors: Hongyan Fu, Ziyao Zheng, Zhenping Zhang, Yanjuan Yang, Jieda Cui, Zhaojun Wang, Jing Xue, Shuhong Chi, Mengshu Cao, Juan Chen

Affiliations

  • Department of Key Laboratory of Ningxia Stem Cell and Regenerative Medicine, Institute of Medical Sciences, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China. Department of Pulmonary and Critical Care Medicine, General Hospital of Xinmi Traditional Chinese Medicine, Xinmi, 452370, Henan, China.
  • Department of Radiology, General Hospital of Ningxia Medical University, Yinchuan, 750004, China.
  • Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China. Department of Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Institute of Human Stem Cell Research, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Department of Rheumatology, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Department of Respiratory and Critical Care Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China.
  • Department of Key Laboratory of Ningxia Stem Cell and Regenerative Medicine, Institute of Medical Sciences, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.
  • Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, 750004, Ningxia, China.

Abstract

OBJECTIVE

Interstitial lung disease (ILD) is a common extramuscular manifestation of the anti-synthetase syndrome (ASS). Patients with ASS-ILD are at risk in developing a progressive fibrosing phenotype despite appropriate treatments. This study investigated the risk factors and the predictive value of multiple risk factors for progressive pulmonary fibrosis (PPF) in patients with ASS-ILD.

METHODS

Ninety patients with a diagnosis of ASS and evidence of ILD on high-resolution computed tomography (HRCT) were recruited. Among them, 72 participants completed follow-up for more than 12 months. These patients were further divided into a PPF-ASS group (n = 18) and a non-PPF-ASS group (n = 54). Logistic regression analysis was performed to investigate the risk factors for PPF. The predictive value of the combined risk factors for predicting PPF were analyzed by a ROC curve.

RESULTS

The PPF-ASS group had a higher rate of positive non-Jo-1 antibodies, a significantly higher neutrophil-to-lymphocyte ratio (NLR) and serum lactate dehydrogenase (LDH), and a significantly lower PaO/FiO ratio and diffusing capacity for carbon monoxide (DLCO%pred) than the non-PPF-ASS group. In addition, elevated serum Krebs von den Lungen-6 (KL-6) level and reticular opacities were significantly more common, and corticosteroid monotherapy at onset was administered more frequently in the PPF-ASS group. The median duration of follow-up was 37.4 months, survival was poorer in the PPF-ASS group, and the overall survival was 88.9%. Multivariate regression analysis further revealed that positive non-Jo-1 antibodies, NLR, and KL-6 were independent risk factors for PPF. These combined indexes had good accuracy (area under the curve = 0.874) in predicting PPF in patients with ASS-ILD.

CONCLUSION

Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors for PPF in patients with ASS-ILD. Monitoring these markers can potentially predict PPF in this group of patients. Key Points • Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors associated with PPF in patients with ASS-ILD. • Monitoring non-Jo-1 antibodies, NLR, and serum KL-6 can potentially predict PPF in patients with ASS-ILD.

MeSH

Ligases, Regression Analysis, Rheumatology, Lung, Serum, Antibodies, Fibrosis, Predictive Value Of Tests, Pulmonary Fibrosis, Risk Factors, Lung Diseases, Lung Diseases, Interstitial

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