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

Li, Defu; Li, Hongbing; Wang, Yujin; Zhu, Tong

doi:10.20452/pamw.16394

Cited by 3 publications

(4 citation statements)

References 31 publications

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“…Xerophthalmia, xerostomia, and dental caries caused by infiltration of exocrine glands by lymphocytes were more common in pSS patients with a long disease duration, which is consistent with the findings of previous studies 7,9 . Meibomian gland dysfunction is an important cause of dry eye 25 . A study indicated that a longer duration of pSS was associated with more severe meibomian gland atrophy after controlling for age, 26 partially explaining the higher proportion of dry eyes in pSS patients with a long disease duration reported in this study.…”

Section: Discussionsupporting

confidence: 92%

“…7,9 Meibomian gland dysfunction is an important cause of dry eye. 25 A study indicated that a longer duration of pSS was associated with more severe meibomian gland atrophy after controlling for age, 26 partially explaining the higher proportion of dry eyes in pSS patients with a long disease duration reported in this study. Furthermore, pSS patients with a long disease duration had higher elasticity scores of parotid glands and a more severe degree of parotid main ductal stenosis.…”

Section: Glandular Injury In Pssmentioning

confidence: 60%

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Disease Duration Affects the Clinical Phenotype of Primary Sjögren Syndrome

Zhang,

Yang,

Chen

et al. 2024

J Clin Rheumatol

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Objectives To investigate the impact of disease duration on clinical phenotypes in Chinese patients with primary Sjögren syndrome (pSS) and examine the correlation between clinical phenotypes and onset age, age at diagnosis, and disease duration. Methods Data from 952 patients diagnosed with pSS in China between January 2013 and March 2022 were analyzed based on medical records. Patients were categorized into 3 groups based on disease duration: short (<5 years), moderate (≥5 and <10 years), and long (≥10 years) group. Clinical characteristics were compared among the 3 groups, and pSS patients with a long disease duration were compared with the other patients after matching age at diagnosis and age at onset. Results Among the patients, 20.4% had a disease duration over 10 years. After matching for age at onset and age at diagnosis, pSS patients with a long disease duration exhibited a significantly higher prevalence of dry mouth (p <0.001), dry eyes (p <0.001), fatigue (p <0.001), arthralgia (p <0.001), and dental caries (p <0.001) and higher rates of anti-Sjögren syndrome A (p < 0.05), anti-Ro52 (p < 0.05), and anti-SSB (p < 0.05) positivity than their control groups, with prevalence increasing with disease duration (p trend < 0.001). However, no differences were noted in the prevalence of interstitial lung disease and leukopenia between different disease duration groups after matching for age at onset, although differences were shown when matching for age at diagnosis. Conclusion Longer disease duration in pSS patients correlates with increased prevalence of sicca symptoms, fatigue, and arthralgia and higher positivity of autoantibodies associated with pSS. However, the prevalence of interstitial lung disease and leukopenia did not correlate with disease duration after matching for age at onset.

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Section: Discussionsupporting

confidence: 92%

Section: Glandular Injury In Pssmentioning

confidence: 60%

Disease Duration Affects the Clinical Phenotype of Primary Sjögren Syndrome

Zhang,

Yang,

Chen

et al. 2024

J Clin Rheumatol

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“…As expected, a significant difference in the relative decline in FVC and DLCO was recorded between progressive and non-progressive ILD. In particular, the median FVC decline was 12% (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22) vs. 0% ((−2.9)-2), for progressive and non-progressive disease, respectively (p < 0.001), and DLCO decline was 9% ((−7.5)-4.25) vs. −1% (2-15.5) for progressive and non-progressive ILD, respectively (p < 0.001).…”

Section: Resultsmentioning

confidence: 98%

“…Some authors reported an association between UIP pattern and increased mortality in RA [5], while in connective tissue diseases (CTDs), patients' radiologic ILD pattern did not modify the survival of the patients [21]. Similarly, radiologic UIP pattern of ILD was not associated with fibrosing progressive ILD in CTDs [22,23], while UIP seems to be progressive in a large percentage of cases in RA-ILD [5,18].…”

Section: Discussionmentioning

confidence: 99%

Fibrosing Progressive Interstitial Lung Disease in Rheumatoid Arthritis: A Multicentre Italian Study

Sebastiani,

Venerito,

Laurino

et al. 2023

JCM

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Background: The INBUILD study demonstrated the efficacy of nintedanib in the treatment of progressive fibrosing interstitial lung disease different to idiopathic pulmonary fibrosis, including rheumatoid arthritis (RA)-related ILD. Nevertheless, the prevalence of RA-ILD patients that may potentially benefit from nintedanib remains unknown. Objectives and methods: The aim of the present multicentre study was to investigate the prevalence and possible associated factors of fibrosing progressive patterns in a cross-sectional cohort of RA-ILD patients. Results: One hundred and thirty-four RA-ILD patients with a diagnosis of RA-ILD, who were confirmed at high-resolution computed tomography and with a follow-up of at least 24 months, were enrolled. The patients were defined as having a progressive fibrosing ILD in case of a relative decline in forced vital capacity > 10% predicted and/or an increased extent of fibrotic changes on chest imaging in a 24-month period. Respiratory symptoms were excluded to reduce possible bias due to the retrospective interpretation of cough and dyspnea. According to radiologic features, ILD was classified as usual interstitial pneumonia (UIP) in 50.7% of patients, nonspecific interstitial pneumonia in 19.4%, and other patterns in 29.8%. Globally, a fibrosing progressive pattern was recorded in 36.6% of patients (48.5% of patients with a fibrosing pattern) with a significant association to the UIP pattern. Conclusion: We observed that more than a third of RA-ILD patients showed a fibrosing progressive pattern and might benefit from antifibrotic treatment. This study shows some limitations, such as the retrospective design. The exclusion of respiratory symptoms’ evaluation might underestimate the prevalence of progressive lung disease but increases the value of results.

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