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Prognostic factors of interstitial lung disease progression at sequential HRCT in anti-synthetase syndrome

  • Hui Liu
  • Sheng XieEmail author
  • Tian Liang
  • Li Ma
  • Hongliang Sun
  • Huaping Dai
  • Chen Wang
Chest
  • 77 Downloads

Abstract

Objectives

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.

Methods

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.

Results

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.

Conclusions

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.

Key Points

• 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.

Keywords

Polymyositis Dermatomyositis Interstitial lung disease Prognosis 

Abbreviations

AIP

Acute interstitial pneumonia pattern

ANA

Anti-nuclear antibody

ASS

Anti-synthetase syndrome

CI

Confidence interval

CK

Creatine kinase

CRP

C-reactive protein

CTDs

Connective tissue diseases

DLCO

Diffusion capacity of the lung for carbon monoxide

ESR

The erythrocyte sedimentation rate

FEV1

Forced expiratory volume

FVC

Forced vital capacity

GGO

Ground glass opacities

HRCT

High-resolution computed tomography

ILD

Interstitial lung disease

NSIP

Nonspecific interstitial pneumonia

OP

Organizing pneumonia

OR

Odds ratio

PFT

Pulmonary function test

RF

Rheumatoid factor

UIP

Usual interstitial pneumonia

VC

Vital capacity

Notes

Acknowledgments

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.

Funding

No financial support was received for this work.

Compliance with ethical standards

Guarantor

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

Informed consent was waived because this was a retrospective study.

Ethical approval

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.

Methodology

• Retrospective

• Diagnostic or prognostic study

• Performed at one institution

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Copyright information

© European Society of Radiology 2019

Authors and Affiliations

  • Hui Liu
    • 1
  • Sheng Xie
    • 1
    Email author
  • Tian Liang
    • 1
  • Li Ma
    • 2
  • Hongliang Sun
    • 1
  • Huaping Dai
    • 3
  • Chen Wang
    • 4
  1. 1.Department of RadiologyChina-Japan Friendship HospitalBeijingChina
  2. 2.Department of RheumatologyChina-Japan Friendship HospitalBeijingChina
  3. 3.Department of PulmonologyBeijing Chaoyang HospitalBeijingChina
  4. 4.Department of PulmonologyChina-Japan Friendship HospitalBeijingChina

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