Prognostic factors of interstitial lung disease progression at sequential HRCT in anti-synthetase syndrome
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Interstitial lung disease (ILD) is a common extra-muscular manifestation of anti-synthetase syndrome (ASS) and the main cause of morbidity and mortality in patients with ASS. Data on prognostic factors in these patients are lacking.
A total of 69 patients with ILD and positivity for at least one of the following autoantibodies were included: anti-Jo-1, anti-PL7, anti-PL12, and anti-EJ. Relevant clinical characteristics were registered. According to the changes in the extent of abnormalities at the follow-up on high-resolution computed tomography (HRCT), three groups were defined: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate possible prognostic factors and multivariate analysis by logistic regression was then applied to determine the independent prognostic factors in ASS-ILD.
The cohort comprised 69 patients positive for anti-synthetase antibodies, i.e., 30 for anti-Jo-1, 16 for anti-EJ, 13 for anti-PL7, and 10 for anti-PL12. The mean length of follow-up was 15 months. Sex, age at diagnosis, fever at presentation, and counts of CD3+CD4+ cells were significantly different among the three groups. According to the multivariate analysis, fever at presentation, lower counts of CD3+CD4+ cells, and a pattern of usual interstitial pneumonia were the three independent risk factors for poor outcomes of ASS-ILD.
At the onset of ASS, some clinical features and HRCT pattern of ILD may suggest an unfavorable outcome of lung involvement on HRCT, even with routine therapy. These factors may contribute to the high long-term mortality of ASS.
• Evaluation of lung involvement on HRCT is important in the follow-up of patients with interstitial lung disease related to anti-synthetase syndrome (ASS-ILD).
• The interstitial lung disease related to ASS responds to the treatment variably.
• Some clinical and imaging characteristics are associated with poor prognosis in patients with ASS-ILD, including fever at diagnosis, a lower serum CD3 + /CD4 + level, and a UIP pattern.
KeywordsPolymyositis Dermatomyositis Interstitial lung disease Prognosis
Acute interstitial pneumonia pattern
Connective tissue diseases
Diffusion capacity of the lung for carbon monoxide
The erythrocyte sedimentation rate
Forced expiratory volume
Forced vital capacity
Ground glass opacities
High-resolution computed tomography
Interstitial lung disease
Nonspecific interstitial pneumonia
Pulmonary function test
Usual interstitial pneumonia
We thank Angela Morben, DVM, ELS, from Liwen Bianji, Edanz Editing China (www.liwenbianji.cn/ac), for editing the English text of a draft of this manuscript.
No financial support was received for this work.
Compliance with ethical standards
The scientific guarantor of this publication is Sheng Xie.
Conflict of interest
The authors declare that they have no conflict of interests.
Statistics and biometry
No complex statistical methods were necessary for this paper.
Informed consent was waived because this was a retrospective study.
The study was approved by the ethical committee in our institution.
Study subjects or cohorts overlap
This study subjects or cohorts was original research that has not been published previously, and not under consideration for publication elsewhere.
• Diagnostic or prognostic study
• Performed at one institution
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