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. 2022 Feb 27;22(1):73.
doi: 10.1186/s12890-022-01868-5.

Interstitial lung disease in Primary Sjögren's syndrome

Wei Lin #  1 , Zhifei Xin #  2 , Jianlong Zhang #  3 , Ning Liu  1 , Xiuying Ren  1 , Meilu Liu  1 , Yashuang Su  1 , Yixuan Liu  1 , Liu Yang  1 , Shaoying Guo  1 , Yupeng Yang  4 , Yang Li  5 , Jingjing Cao  1 , Xiaoran Ning  1 , Jingjing Li  1 , He Xue  1 , Nannan Niu  1 , Yingmin Chen  3 , Fang Li  1 , Lijun Sun  6 , Xiaopeng Zhang  7 , Fengxiao Zhang  1 , Wen Zhang  8
Affiliations

Interstitial lung disease in Primary Sjögren's syndrome

Wei Lin et al. BMC Pulm Med. .

Abstract

Background: Interstitial lung disease (ILD) may cause life-threatening complications of primary Sjogren's syndrome (pSS), and has a poor prognosis in terms of survival and quality of life. To date, few studies have investigated the risk factors for ILD detected by high-resolution computed tomography (HRCT) in pSS patients with or without respiratory symptoms.

Methods: Data of 333 patients with newly diagnosed pSS were retrospectively analysed. Interstitial lung disease involvement was defined as typical abnormalities on HRCT and/or pulmonary function tests. Multivariate regression model was used to evaluate the association between interstitial lung disease and pSS characteristics.

Results: Sixty-six patients (19.82%) were diagnosed with pSS-ILD. Ground glass opacities (87.88%) and septal/sub pleural lines (81.82%) were most frequent. Based on pulmonary high-resolution computed tomography, patients were divided into nonspecific (n = 42), usual (n = 20), lymphocytic interstitial pneumonia (n = 3) and cryptogenic organising pneumonia (n = 1) groups. There was a strong association between erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) and the HRCT-score. Pulmonary function tests revealed impaired diffusion capacity for carbon monoxide and total lung capacity, and coexistence of small airway lesions in pSS-interstitial lung disease. On logistic regression analysis, age, Raynaud's phenomenon, lymphopenia, cough, dyspnoea and rampant dental caries were risk factors associated with pSS-interstitial lung disease.

Conclusions: Interstitial lung disease involvement in pSS is a common clinical occurrence. The clinical manifestation is nonspecific and variable; Raynaud's phenomenon and lymphopenia may predict its onset. pSS patients with advanced age, dry cough and dyspnoea should be systematically evaluated for ILD involvement and managed according to their symptoms.

Keywords: Interstitial lung disease; Primary Sjögren syndrome; Raynaud's phenomenon; Risk factors; Warrick score.

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Conflict of interest statement

The authors declare no potential conflicts of interest.

Figures

Fig. 1
Fig. 1
Extraglandular involvements in pSS patients with and without ILD
Fig. 2
Fig. 2
Correlation between the HRCT-score and PFT results in pSS-ILD patients
Fig. 3
Fig. 3
Multivariate analysis of features predicting ILD in pSS patients. CI Confidence interval, OR odds ratio
See this image and copyright information in PMC

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