Prediction of Progressive Pulmonary Fibrosis in Patients with Anti-synthetase Syndrome-associated Interstitial Lung Disease

Overview

Journal Clin Rheumatol

Publisher Springer

Specialty Rheumatology

Date 2023 Mar 17

PMID 36929316

Authors

Hongyan Fu

Ziyao Zheng

Zhenping Zhang

Yanjuan Yang

Jieda Cui

Zhaojun Wang

Jing Xue

Shuhong Chi

Mengshu Cao

Juan Chen

Affiliations

Soon will be listed here.

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.

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