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

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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.

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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

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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.

Author information

Authors and Affiliations

  1. Department of Radiology, China-Japan Friendship Hospital, Beijing, 100029, China

    Hui Liu, Sheng Xie, Tian Liang & Hongliang Sun

  2. Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China

    Li Ma

  3. Department of Pulmonology, Beijing Chaoyang Hospital, Beijing, 100029, China

    Huaping Dai

  4. Department of Pulmonology, China-Japan Friendship Hospital, Beijing, 100029, China

    Chen Wang

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  1. Hui Liu
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Corresponding author

Correspondence to Sheng Xie.

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The scientific guarantor of this publication is Sheng Xie.

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The authors declare that they have no conflict of interests.

Statistics and biometry

No complex statistical methods were necessary for this paper.

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Informed consent was waived because this was a retrospective study.

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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.

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• Retrospective

• Diagnostic or prognostic study

• Performed at one institution

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Liu, H., Xie, S., Liang, T. et al. Prognostic factors of interstitial lung disease progression at sequential HRCT in anti-synthetase syndrome. Eur Radiol 29, 5349–5357 (2019). https://doi.org/10.1007/s00330-019-06152-5

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  • DOI: https://doi.org/10.1007/s00330-019-06152-5

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