The Increased Ratio of Blood CD56<sup>bright</sup> NK to CD56<sup>dim</sup> NK Is a Distinguishing Feature of Primary Sjögren’s Syndrome

Ming, Bingxia; Wu, Tong; Cai, Shaozhe; Hu, Peng; Tang, Jungen; Zheng, Fang; Ye, Cong; Dong, Lingli

doi:10.1155/2020/7523914

Cited by 23 publications

(25 citation statements)

References 32 publications

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“…The main reason for the marked CD4 + T-cell lymphopenia in different age-onset pSS patients needs to be further studied. Interestingly, we also found CD16/CD56+ NK cells, innate lymphoid cells that exhibit a potential regulatory role in exocrine gland tissues and the peripheral blood of pSS disease [ 17 , 18 ] were significantly decreased in the early-onset group. Our data provide evidence that the immunological status may contribute to the distinct features of different age-onset pSS patients.…”

Section: Discussionmentioning

confidence: 72%

Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

et al. 2021

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Objectives To further investigate the clinical characteristics and circulating lymphocyte profiles of patients with early-onset primary Sjögren’s syndrome (pSS). Method Data of 333 patients with pSS were analyzed retrospectively. Early onset was defined as a pSS diagnosis at an age of 35 years or younger. The clinical, laboratory, and immunophenotypic profiles of peripheral blood lymphocyte subsets were compared between early- and later-onset pSS. Results Thirty-six (10.81%) patients matched the definition of early-onset pSS, with age at disease onset being 28.97 ± 5.53 years. Elevated serum IgG level (77.14% vs 31.16%, p < 0.001), low C3 (41.67% vs 20.20%, p = 0.004) and C4 levels (27.78% vs 6.40%, p < 0.001), anti-SSA positivity (91.67% vs 51.85%, p < 0.001), and anti-SSB positivity (50% vs 20.54%, p < 0.001) were more frequent in early-onset patients. The frequencies of hematological (80.56% vs 52.53%, p = 0.001), renal (19.44% vs 5.05%, p = 0.005), and mucocutaneous involvement (50% vs 22.56%, p < 0.001) were significantly higher in the early-onset pSS group, which showed a higher ESSDAI (11(6.25-17) vs 7(3-12); p = 0.003), compared with the later-onset group. In addition, profound CD4+ T-cell lymphopenia was found in patients with early-onset. Conclusions Patients with early-onset pSS have distinctive clinical manifestations and greater activation of the cellular immune system, present with more severe clinical symptoms and immunological features, have increased activation of circulating T cells, and have an unfavorable prognosis. Thus, they require more positive treatment with glucocorticoids and/or immunosuppressants and merit closer follow-up and regular monitoring.

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Section: Discussionmentioning

confidence: 72%

Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

et al. 2021

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“…The relative and total numbers of circulating NK cells and CD56 dim NK cells were lower while the number of CD56 bright NK cells was higher in pSS patients compared to healthy control subjects (67,68). The ratio of CD56 bright to CD56 dim NK cells was correlated with IgG level and showed diagnostic utility for pSS with good sensitivity and specificity (67). The lower numbers of circulating NK cells and CD56 dim NK cells in pSS patients may lead to their infiltration into glands and focal immune injury.…”

Section: Nk Cells and Sjögren's Syndromementioning

confidence: 79%

“…However, recent findings have suggested that innate immunity-especially NK cells-plays a role in SS pathogenesis (64)(65)(66). The relative and total numbers of circulating NK cells and CD56 dim NK cells were lower while the number of CD56 bright NK cells was higher in pSS patients compared to healthy control subjects (67,68). The ratio of CD56 bright to CD56 dim NK cells was correlated with IgG level and showed diagnostic utility for pSS with good sensitivity and specificity (67).…”

Section: Nk Cells and Sjögren's Syndromementioning

confidence: 99%

NK Cells in Autoimmune Diseases: Protective or Pathogenic?

2021

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Autoimmune diseases generally result from the loss of self-tolerance (i.e., failure of the immune system to distinguish self from non-self), and are characterized by autoantibody production and hyperactivation of T cells, which leads to damage of specific or multiple organs. Thus, autoimmune diseases can be classified as organ-specific or systemic. Genetic and environmental factors contribute to the development of autoimmunity. Recent studies have demonstrated the contribution of innate immunity to the onset of autoimmune diseases. Natural killer (NK) cells, which are key components of the innate immune system, have been implicated in the development of multiple autoimmune diseases such as systemic lupus erythematosus, type I diabetes mellitus, and autoimmune liver disease. However, NK cells have both protective and pathogenic roles in autoimmunity depending on the NK cell subset, microenvironment, and disease type or stage. In this work, we review the current knowledge of the varied roles of NK cell subsets in systemic and organic-specific autoimmune diseases and their clinical potential as therapeutic targets.

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“…We noted a weak negative correlation between the IFN score and the fraction of NK cells. The role of NK cells in pSS pathogenesis is unclear, and reduced NK cell numbers and activity in pSS have been reported, as well as a shift within the NK cell sub-populations (32,33). The reasons behind the negative correlation observed is unclear and needs to be further explored.…”

Section: Discussionmentioning

confidence: 98%

DNA Methylation-Based Interferon Scores Associate With Sub-Phenotypes in Primary Sjögren’s Syndrome

et al. 2021

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Primary Sjögren’s syndrome (pSS) is an autoimmune inflammatory disease with profound clinical heterogeneity, where excessive activation of the type I interferon (IFN) system is considered one of the key mechanisms in disease pathogenesis. Here we present a DNA methylation-based IFN system activation score (DNAm IFN score) and investigate its potential associations with sub-phenotypes of pSS. The study comprised 100 Swedish patients with pSS and 587 Swedish controls. For replication, 48 patients with pSS from Stavanger, Norway, were included. IFN scores were calculated from DNA methylation levels at the IFN-induced genes RSAD2, IFIT1 and IFI44L. A high DNAm IFN score, defined as > meancontrols +2SDcontrols (IFN score >4.4), was observed in 59% of pSS patients and in 4% of controls (p=1.3x10-35). Patients with a high DNAm IFN score were on average seven years younger at symptom onset (p=0.017) and at diagnosis (p=3x10-3). The DNAm IFN score levels were significantly higher in pSS positive for both SSA and SSB antibodies compared to SSA/SSB negative patients (pdiscovery=1.9x10-8, preplication=7.8x10-4). In patients positive for both SSA subtypes Ro52 and Ro60, an increased score was identified compared to single positive patients (p=0.022). Analyzing the discovery and replication cohorts together, elevated DNAm IFN scores were observed in pSS with hypergammaglobulinemia (p=2x10-8) and low C4 (p=1.5x10-3) compared to patients without these manifestations. Patients < 70 years with ongoing lymphoma at DNA sampling or lymphoma at follow-up (n=7), presented an increased DNAm IFN score compared to pSS without lymphoma (p=0.025). In conclusion, the DNAm-based IFN score is a promising alternative to mRNA-based scores for identification of patients with activation of the IFN system and may be applied for patient stratification guiding treatment decisions, monitoring and inclusion in clinical trials.

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The Increased Ratio of Blood CD56^bright NK to CD56^dim NK Is a Distinguishing Feature of Primary Sjögren’s Syndrome

Cited by 23 publications

References 32 publications

Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

NK Cells in Autoimmune Diseases: Protective or Pathogenic?

DNA Methylation-Based Interferon Scores Associate With Sub-Phenotypes in Primary Sjögren’s Syndrome

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The Increased Ratio of Blood CD56bright NK to CD56dim NK Is a Distinguishing Feature of Primary Sjögren’s Syndrome

Cited by 23 publications

References 32 publications

Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles

NK Cells in Autoimmune Diseases: Protective or Pathogenic?

DNA Methylation-Based Interferon Scores Associate With Sub-Phenotypes in Primary Sjögren’s Syndrome

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The Increased Ratio of Blood CD56^bright NK to CD56^dim NK Is a Distinguishing Feature of Primary Sjögren’s Syndrome