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系统性硬化症患者中干燥综合征和口干症状的患病率以及小唾液腺活检标本中α平滑肌肌动蛋白的表达

The prevalence of Sjögren’s syndrome and sicca symptoms in patients with systemic sclerosis and alpha-smooth muscle actin expression in biopsy specimens from minor salivary glands.

作者信息

Can Gerçek, Sarıoğlu Sülen, Birlik Merih, Kenar Gökçe, Soysal Özgül, Solmaz Dilek, Gerdan Vedat, Önen Fatoş, Akkoç Nurullah, Akar Servet

机构信息

Department of Rheumatology, Dokuz Eylül University School of Medicine, İzmir, Turkey

Department of Pathology, Dokuz Eylül University School of Medicine, İzmir, Turkey

出版信息

Turk J Med Sci. 2021 Aug 30;51(4):1875-1882. doi: 10.3906/sag-2012-25.

Abstract

BACKGROUND/AIM: This study aimed to investigate the prevalence of sicca symptoms and secondary Sjögren’s syndrome (SjS) in patients with systemic sclerosis (SSc). Also this study aimed to evaluate the expression of α-smooth muscle actin (α–SMA) in minor salivary gland (MSG) specimens, a possible marker of fibrosis responsible for myofibroblastic transformation.

MATERIALS AND METHODS

Patients with SSc who were followed in Rheumatology outpatient clinic at a university hospital evaluated. The questionnaire of sicca symptoms and classification of SjS were evaluated according to the American–European Consensus Group (AECG) criteria. Histopathologic evaluations were done in MSG specimens investigating the presence of focal lymphocytic sialadenitis and glandular fibrosis, also assessing the expression of α–SMA.

RESULTS

This cross-sectional study included 102 patients with SSc [91 females (89%), mean age 52.5 ± 12 years]. In this cohort 76 (75%) patients had sicca symptoms and 36 (35.3%) patients fulfilled the AECG criteria for SjS; all with limited form. Having SjS found to be associated with older age and the presence of positive anti-SS-A antibodies. On histopathologic examinations, glandular fibrosis was observed in 67 (80%) and lymphocytic sialadenitis was detected in 38 (45%) patients; but only 7 samples were positive for α–SMA.

CONCLUSION

This study suggested sicca symptoms were found to be very common among patients with SSc. Also secondary SjS was detected in nearly one-third of patients with SSc; especially in limited subtype. Anti SS-A positivity and older age were detected as predictors for SjS. Histopathologic evaluations showed significant glandular fibrosis but rare α-SMA staining in patients with SSc.

摘要

背景/目的:本研究旨在调查系统性硬化症(SSc)患者中口干症状和继发性干燥综合征(SjS)的患病率。此外,本研究旨在评估小唾液腺(MSG)标本中α平滑肌肌动蛋白(α–SMA)的表达,α–SMA是负责肌成纤维细胞转化的纤维化的一种可能标志物。

材料与方法

对在大学医院风湿病门诊随访的SSc患者进行评估。根据欧美共识小组(AECG)标准评估口干症状问卷和SjS分类。对MSG标本进行组织病理学评估,调查局灶性淋巴细胞性涎腺炎和腺纤维化的存在情况,并评估α–SMA的表达。

结果

这项横断面研究纳入了102例SSc患者[91例女性(89%),平均年龄52.5±12岁]。在该队列中,76例(75%)患者有口干症状,36例(35.3%)患者符合SjS的AECG标准;均为局限性类型。发现患有SjS与年龄较大和抗SS-A抗体阳性有关。组织病理学检查显示,67例(80%)患者有腺纤维化,38例(45%)患者检测到淋巴细胞性涎腺炎;但只有7份样本α–SMA呈阳性。

结论

本研究表明,口干症状在SSc患者中非常常见。此外,在近三分之一的SSc患者中检测到继发性SjS;尤其是在局限性亚型中。检测到抗SS-A阳性和年龄较大是SjS的预测因素。组织病理学评估显示SSc患者有明显的腺纤维化,但α-SMA染色少见。

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