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Pulmonary Manifestations of Primary Sjogren's Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management.

ABSTRACT: Pulmonary involvement in primary Sjögren's syndrome (pSS) is an understudied entity with important clinical implications. Its prevalence has been reported in up to 20% of pSS patients. Pulmonary manifestations of pSS are diverse with involvement of airway and/or lung parenchyma. Histopathology of lung lesions suggests a predominance of submucosal mononuclear cell infiltration consisting predominantly of CD4+ T cells. Current understanding of the pathophysiology of lung disease in pSS suggests a similar process driving the pulmonary process as those in the salivary glands, with epithelial cells playing a critical role in the initiation, maintenance, and symptomatology of the disease. Clinical manifestations of lung involvement in pSS are as varied as the underlying pathology and can be vague and non-specific, thus delaying diagnosis. Management options depend on the underlying pathology but are generally limited due to lack of systematic randomized controlled trials. This review helps summarize our current understanding of lung involvement in pSS.

SUBMITTER: Gupta S

PROVIDER: S-EPMC6583234 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Pulmonary Manifestations of Primary Sjögren's Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management.

Gupta Sarthak S Ferrada Marcela A MA Hasni Sarfaraz A SA

Frontiers in immunology 20190612

Pulmonary involvement in primary Sjögren's syndrome (pSS) is an understudied entity with important clinical implications. Its prevalence has been reported in up to 20% of pSS patients. Pulmonary manifestations of pSS are diverse with involvement of airway and/or lung parenchyma. Histopathology of lung lesions suggests a predominance of submucosal mononuclear cell infiltration consisting predominantly of CD4+ T cells. Current understanding of the pathophysiology of lung disease in pSS suggests a ...[more]

PMID: 31249572

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Project description:BackgroundPrimary Sjögren's syndrome (pSS) is an autoimmune condition that causes harm to exocrine glands and also has extra-glandular manifestations (EGM). pSS patients with EGM have a worse prognosis than those with only sicca symptoms. Previous studies have shown that the minor salivary glands (MSG) of pSS patients exhibit a unique profile of cytokines and chemokines compared to healthy controls. However, there is a lack of research comparing pSS with EGM (pSS-EGM) and pSS without EGM (pSS-non-EGM). This study aims to explore potential biomarkers associated with pSS, particularly pSS with EGM.MethodsBy utilizing RNA sequencing, we conducted an analysis on the gene expression profiles of MSG in 63 patients diagnosed with pSS, as well as 12 non-pSS individuals. Furthermore, we also investigated the MSG of pSS patients, both with and without EGM. Through bioinformatics analysis, we identified genes with differential expression (DEGs) and determined the core hub genes using PPI network. We then analyzed the top 20 DEGs and their correlation with the patients' clinical characteristics, and validated our findings using peripheral blood plasma.ResultsA total of 725 differentially expressed genes (DEGs) were identified in the comparison between pSS and non-pSS groups, and 727 DEGs were observed between pSS-EGM and pSS-non-EGM. It is noteworthy that the expression levels of CXCL9 were higher in both pSS patients and pSS-EGM when compared to the control group. Taking into consideration the significance of the top 20 DEGs in relation to clinical parameters and the central hub genes, we ultimately chose CXCL9. In comparison to the non-pSS group, pSS patients exhibited notably greater expression of the CXCL9 gene in the MSG, as well as higher levels of CXCL9 protein in their plasma (p < 0.001). Furthermore, the expression of the CXCL9 gene and levels of CXCL9 protein were notably higher in pSS patients accompanied by EGM and those with SSA antibodies. Additionally, a correlation was found between the expression of the CXCL9 gene and the EULAR Sjogren's Syndrome Disease Activity Index (ESSDAI), as well as with immunoglobulin G (IgG) levels and erythrocyte sedimentation rate (ESR). Meanwhile, the protein levels of CXCL9 were found to be correlated with IgG levels and ESSDAI.ConclusionCXCL9 proves to be a valuable biomarker in pSS, specifically due to its strong ability to differentiate between pSS patients with EGM and those without EGM. There is a significant correlation between CXCL9 and various clinical parameters both at the gene and protein level. Therefore, CXCL9 could be a potential target for future treatment of pSS.

Dataset Information

Pulmonary Manifestations of Primary Sjogren's Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management.

Publications

Pulmonary Manifestations of Primary Sjögren's Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management.

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