Clinical profiles of SS-ILD compared with SS-NILD in a Chinese population: a retrospective analysis of 735 patients

Abstract

Background Interstitial lung disease (ILD) is a serious complication in patients with Sjögren’s syndrome (SS). Most studies on primary SS (pSS) with ILD are limited in sample size, and studies on secondary SS (sSS) with ILD are rare. This study aimed to elucidate both primary and secondary SS-associated ILD (SS-ILD) based on a large cohort. Methods The medical records of hospitalized patients diagnosed with SS at the Second Xiangya Hospital of Central South University from January 2010 to May 2020 were retrospectively reviewed. Clinical manifestations, medical history, biological results and imaging data were collected. Results Of the 735 SS patients enrolled in this study, 563 (76.6%) were diagnosed with pSS, 172 (23.4%) were diagnosed with sSS. Additionally, 316 (43.0%) were diagnosed with SS-ILD. No significant difference was found between the pSS and sSS groups concerning the incidence of ILD ( p = .718). Factors associated with SS-ILD were older age ( p < .001), male sex ( p = .032), female sex at menopause ( p = .002), Raynaud’s phenomenon ( p < .001), low levels of albumin ( p = .010) and respiratory symptoms ( p < .001). The SS-ILD group showed higher counts of platelets ( p < .001). The three most frequent high-resolution CT (HRCT) findings of SS-ILD were irregular linear opacities (42.7%), grid shadows (30.7%) and pleural thickening (28.5%). NSIP (56.3%) was the most frequent HRCT pattern. Compared with pSS patients with ILD (pSS-ILD) patients, sSS patients with ILD (sSS-ILD) patients had a higher incidence of proteinuria ( p < .001) and hypercreatinaemia ( p = .013), a higher level of erythrocyte sedimentation rate (ESR) ( p = .003), low levels of complement 3 (C3) ( p = .013), lymphocytes ( p = .009) and leukocytes ( p = .024), and worse DLCO (%Pred) ( p = .035). Conclusions ILD is a common pulmonary involvement in both pSS patients and sSS patients. Older age, male sex, female sex at menopause, Raynaud’s phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. NSIP is important HRCT feature of SS-ILD. sSS-ILD patients showed worse laboratory results and pulmonary function. KEY MESSAGE Older age, male sex, female sex at menopause, Raynaud’s phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. SS-ILD patients show higher counts of platelets and less purpura. sSS-ILD patients have worse laboratory results and pulmonary functi...

Clinical course and risk factors for development and progression of interstitial lung disease in primary Sjögren’s syndrome

“…High-resolution computed tomography is the most sensitive imaging tool and gold standard for the diagnosis of ILDs. Previous studies focusing on CT patterns showed that non-specific interstitial pneumonia (NSIP) was the most frequent pattern in pSS-ILD 10 , and the usual interstitial pneumonia (UIP) pattern was a determinant of progression and mortality 11 . Furthermore, HRCT can provide quantitative information on the extent and severity of the disease, as well as pattern analysis.…”

Pulmonary fibrosis in primary Sjögren’s syndrome: computed tomography, clinical features, and associated clinical factors

“…Numerous studies have suggested that the risk factors for Sjögren's syndrome (SS)related ILD include age, smoking status, male sex, antinuclear antibody (ANA) positivity, rheumatoid factor (RF) level, C-reactive protein (CRP) level, respiratory symptoms, a postmenopausal period in women, low albumin levels, Raynaud's syndrome, lymphopenia, and rampant caries [7][8][9][10][11]. Our recent findings suggest that pSS patients with positive anti-Ro52 antibodies have a lower risk of developing pulmonary fibrosis than those with negative anti-Ro52 antibodies (OR: 0.419, 95% CI: 0.183-0.963) [12].…”

Clinical significance of anti-rheumatoid arthritis 33 antibody in patients with systemic lupus erythematosus

“…Previous studies have revealed an association between the occurrence of ILD and Raynaud's phenomenon in patients with CTDs, such as pSS, SLE, and MCTD. [47][48][49] Notably, a higher percentage of patients with Raynaud's phenomenon was found in the RA33-positive group, which may at least partially explain the higher proportion of ILD in CTD patients with anti-RA33 positivity in this study. Notably, previous studies have shown a remarkable correlation between the positivity of anti-RA33 and anti-snRNP antibodies.…”