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Risk factors and prognosis of interstitial lung disease for primary Sjögren syndrome patients: A retrospective case‒control study

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Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Objective

To determine the prevalence, clinical features, risk factors, and prognosis of interstitial lung disease (ILD) in patients with primary Sjogren's syndrome (pSS).

Methods

Data from 274 pSS patients from August 2013 to August 2022 were reviewed. The clinical features of pSS with ILD were revealed. Logistic regression was used to determine risk factors for ILD in pSS patients. Survival analysis and Cox regression were used to analyse the prognosis and prognostic factors of pSS patients.

Results

In pSS patients, the prevalence of ILD was 22.3% (61/274). The pSS patients with ILD were characterized by a late onset and long disease course, with a nonspecific interstitial pneumonia (NSIP) pattern as the predominant high-resolution computed tomography (HRCT) finding. Logistic regression results indicated that an age over 50 years old (OR 4.786, 95% CI 1.602–14.299; P = 0.005), purpuric rash (OR 4.695, 95% CI 1.537–14.339; P = 0.007), AMA-M2 antibody positivity (OR 2.582, 95% CI 1.166–5.722; P = 0.019), and diabetes (OR 2.514, 95% CI 1.025–6.167; P = 0.044) were risk factors for ILD in pSS patients. Cox regression results showed that advanced age (HR 1.240, 95% CI 1.088–1.413; P = 0.001) and cancer history (HR 8.411, 95% CI 1.771–39.934; P = 0.007) were risk factors for pSS patient survival.

Conclusion

This study showed that pSS patients with ILD tended to have a late onset and long course of pSS. An age over 50 years, purpuric rash, AMA-M2 antibody positivity, and diabetes were risk factors for ILD in pSS patients. Advanced age and cancer history were prognostic factors in pSS patients.

Key Points

• This study showed that pSS patients with ILD tended to have a late-onset and lengthy course of pSS, with the NSIP pattern as the predominant lung image.

• The risk factors for ILD in pSS patients determined in this study were an age over 50 years, purpuric rash, AMA-M2 antibody positivity, and diabetes.

• The prognostic risk factors for pSS patients were advanced age and cancer history.

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Data availability

All data generated or analysed during this study are included in this published article.

Abbreviations

ILD:

Interstitial lung disease

pSS:

Primary Sjogren's syndrome

pSS-ILD:

Primary Sjögren’s syndrome-associated interstitial lung disease

pSS-nonILD:

Primary Sjögren’s syndrome without interstitial lung disease

CTD:

Connective tissue disease

HRCT:

High-resolution computed tomography

NSIP:

Nonspecific interstitial pneumonia

UIP:

Usual interstitial pneumonia

OP:

Organizing pneumonia

LIP:

Lymphocytic interstitial

ESR:

Erythrocyte sedimentation rate

CRP:

C-reactive protein

IgG:

Immunoglobulin G

WBC:

White blood cell count

PLT:

Platelet count

ESSDAI:

EULAR primary Sjögren syndrome Disease Activity Index

PFT:

Pulmonary function test

FVC%:

Forced vital capacity percent predicted

Dlco%:

Diffusion capacity CO percent predicted

ANA:

Anti-nuclear antibody

Anti-SSA :

Anti-Sjogren's syndrome A

Anti-SSB:

Anti-Sjogren's syndrome B

AMA-M2:

Anti-mitochondrial M2

Anti-RNP:

Anti-ribonucleoprotein

GERD:

Gastroesophageal reflux disease

COPD:

Chronic obstructive pulmonary disease

AIH:

Autoimmune hepatitis

PBC:

Primary biliary cirrhosis

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Funding

This research was supported by the Natural Science Foundation of Zhejiang Province, China (Y21H270014), the TCM Science and Technology Program of Zhejiang Province, China (2020ZZ006), and the Scientific Research Project of Zhejiang Provincial Education Department, China (Y202146162).

Author information

Author notes

  1. Yichen Huang, Yanran Qiu, Zhijun Xie these authors contributed equally to this work.

Authors and Affiliations

  1. The Second Clinical College, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China

    Yichen Huang, Yanran Qiu, Meihui Guan, Xinchang Wang, Wanru Cai & Jing Sun

  2. School of Basic Medical Science, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China

    Zhijun Xie, Fengqi Zhang & Yi Zhang

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  1. Yichen Huang
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Contributions

Y.H, Y.Q., and Z.X. designed this study. M.G. was involved in the case collection, data analysis, and patient follow-up. Y.H. and Y.Q. finished the article writing. F.Z., Y.Z., and Z.X. revised the manuscript. W.C., X.W., and J.S. supervised this study and reviewed the article, contributing equally to this work as co-correspondence authors. All authors approved the submission of the final version of the manuscript.

Corresponding authors

Correspondence to Xinchang Wang, Wanru Cai or Jing Sun.

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None declared.

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No additional data were available.

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Patients and the public were involved in the design and conception of the study, and there are plans to disseminate the results to patients.

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Huang, Y., Qiu, Y., Xie, Z. et al. Risk factors and prognosis of interstitial lung disease for primary Sjögren syndrome patients: A retrospective case‒control study. Clin Rheumatol 42, 3033–3041 (2023). https://doi.org/10.1007/s10067-023-06596-7

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