Characteristics of patients with primary Sjögren’s syndrome associated interstitial lung disease and relevant features of disease progression
Clinical Rheumatology(2020)
Abstract
Objective To investigate characteristics of patients with primary Sjögren’s syndrome (pSS)-associated interstitial lung disease (ILD) and relevant features of ILD progression. Method Patients with pSS were retrospectively reviewed, and pSS-ILD and pSS non-ILD were identified. Clinical data, laboratory parameters, pulmonary high-resolution CT (HRCT), and pulmonary function tests (PFTs) were collected. pSS-ILD patients were further categorized into subgroups according to HRCT patterns or PFTs. Results Eighty-five pSS-ILD patients and 85 pSS non-ILD patients were included. The average age at disease onset and median disease duration were significantly higher in pSS-ILD patients than those in pSS non-ILD patients ( p < 0.001). Fever, xerostomia, xerophthalmia, and numbness were more frequent, and white blood cells, C reactive protein, and immunoglobulin G (IgG) levels were higher in pSS-ILD patients when compared to pSS non-ILD patients ( p < 0.01). More male patients, older age at disease onset, and less frequent anti-Ro52 antibody were noted in patients with CT-usual interstitial pneumonia (UIP) pattern. In 49 patients with pSS-ILD, who repeated PFTs 6 months from the baseline, 79.6% were stable while 20.4% progressed, with ESR and CT-UIP pattern related with disease progression. Conclusions Patients with pSS-ILD were characterized by more frequent fever, xerophthalmia, and elevated IgG levels, while male, older age at disease onset, and less frequent anti-Ro52 antibody were related with CT-UIP pattern. ESR and CT-UIP pattern were potential predictors for ILD progression. Key Points • pSS-ILD patients are characterized by more frequent fever, xerophthalmia and elevated IgG . • Anti-Ro52 antibody is less frequent in patients with CT-UIP pattern compared to non-UIP patterns . • ESR and CT-UIP pattern are associated with pSS-ILD progression .
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Key words
Interstitial lung disease,Primary Sjögren’s syndrome,Usual interstitial pneumonia
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