Ercole Linete Parolin, Malvezzi Mariester, Boaretti Antonio Carlos, Utiyama Shirley Ramos, Rachid Acir
Department of Medicine, Hospital de Clínicas of the Federal University of Paraná, Curitiba, Paraná, Brazil.
J Investig Allergol Clin Immunol. 2003;13(2):87-93.
Systemic sclerosis (SSc) is a chronic inflammatory connective-tissue disease of unknown etiology, characterized by fibrosis and microvascular injury in affected organs. It has become clear that the activated cellular-immune system plays a central role in the pathogenesis of SSc. This study analyzes the numbers of lymphocyte subpopulations and their relations with clinical and laboratory manifestations. We studied a group of 42 patients with SSc and a group of 28 matched normal controls by flow cytometry using the lymphocyte cell-surface markers CD2, CD3, CD4, CD8, CD19, CD25, CD45RA, CD56, CD71, HLA-DR, TCR alpha/beta, and TCR gamma/delta. Patients with SSc had similar percentages of CD2+, CD3+, CD3+ CD4+, CD3+, CD8+, CD25+, CD4+ CD45RA+, CD8+ CD45RA+, CD71+ cells, and CD4+/CD8+ cell ratio when compared to normal controls. In contrast, the percentages of TCR gamma/delta cells were significantly lower in SSc patients with diffuse and late-stage disease with pulmonary involvement, muscle involvement, and the presence of anti-Scl-70 antibodies. Patients with diffuse SSc in early- and late-stage disease had significantly increased percentages of HLA-DR in CD4+ and CD8+ cells. Patients with late-stage disease had increased percentages of CD4+ CD45RA+ T-cells. Patients with limited and early-stage disease had smaller percentages of B-cells (CD19+). Patients with diffuse and late-stage disease had smaller percentages of NK-cells (CD56+). These results suggest that T-, B-, and NK-cell alterations may be involved in the onset of the disease, and/or in the perpetuation of disease, and may eventually be useful as a prognostic indicator in selected patient subgroups.
系统性硬化症（SSc）是一种病因不明的慢性炎症性结缔组织疾病，其特征是受累器官出现纤维化和微血管损伤。目前已经明确，激活的细胞免疫系统在SSc的发病机制中起核心作用。本研究分析了淋巴细胞亚群的数量及其与临床和实验室表现的关系。我们通过流式细胞术，使用淋巴细胞表面标志物CD2、CD3、CD4、CD8、CD19、CD25、CD45RA、CD56、CD71、HLA-DR、TCRα/β和TCRγ/δ，对42例SSc患者和28例匹配的正常对照进行了研究。与正常对照相比，SSc患者的CD2 +、CD3 +、CD3 + CD4 +、CD3 +、CD8 +、CD25 +、CD4 + CD45RA +、CD8 + CD45RA +、CD71 +细胞百分比以及CD4 + / CD8 +细胞比值相似。相比之下，在患有弥漫性和晚期疾病且有肺部受累、肌肉受累以及抗Scl - 70抗体阳性的SSc患者中，TCRγ/δ细胞的百分比显著降低。早期和晚期弥漫性SSc患者的CD4 +和CD8 +细胞中HLA-DR的百分比显著增加。晚期疾病患者的CD4 + CD45RA + T细胞百分比增加。局限性和早期疾病患者的B细胞（CD19 +）百分比更小。弥漫性和晚期疾病患者的NK细胞（CD56 +）百分比更小。这些结果表明，T细胞、B细胞和NK细胞的改变可能参与了疾病的发生和/或疾病的持续发展，最终可能作为特定患者亚组的预后指标。
