Fernández-Cladera Yolanda, García-González María, Hernández-Díaz Marta, Gómez-Bernal Fuensanta, Quevedo-Abeledo Juan C, González-Rivero Agustín F, de Vera-González Antonia, Gómez-Moreno Cristina, González-Gay Miguel Á, Ferraz-Amaro Iván
Division of Central Laboratory, Hospital Universitario de Canarias, 38320 Tenerife, Spain.
Division of Rheumatology, Hospital Universitario de Canarias, 38320 Tenerife, Spain.
Biomedicines. 2024 Apr 27;12(5):967. doi: 10.3390/biomedicines12050967.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder identified by hematological abnormalities including anemia, leukopenia, and thrombocytopenia. Complement system disturbance is implicated in the pathogenesis of SLE. In this work, we aim to study how a full assessment of the complement system, which includes the evaluation of its three pathways, relates to blood cell counts in a population of patients with SLE. New-generation functional assays of the classical, alternative, and lectin pathways of the complement system were conducted in 284 patients with SLE. Additionally, serum levels of inactive molecules (C1q, C2, C3, C4, factor D) and activated molecules (C3a), as well as regulators (C1-inhibitor and factor H), were evaluated. Complete blood cell counts were analyzed. Multivariable linear regression analysis was performed to study the relationship of hematological profiles with this full characterization of the complement system. After multivariable adjustments that included age, sex, SLICC-DI (damage), and SLEDAI (activity) scores, as well as the use of aspirin, prednisone, methotrexate, azathioprine, and mycophenolate mofetil, several relationships were observed between the C pathways and the individual products and blood cells profile. Lower values of C1q and C2 were associated with lower hemoglobin levels. Lower leukocyte counts showed significantly lower values of C4, C1 inhibitor, C3, factor D, and alternative pathway functional levels. Neutrophil counts showed significant negative relationships only with the alternative pathway and C1-inh. In the case of lymphocytes, associations were found, especially with functional tests of the classical and alternative pathways, as well as with C2, C4, C3, and C3a. On the contrary, for platelets, significance was only observed, after multivariable adjustment, with lower C2 concentrations. In conclusion, the serum complement system and hematological profile in SLE are independently linked, after adjustment for disease activity and damage. These relationships are basically negative and are predominantly found in lymphocytes.
系统性红斑狼疮（SLE）是一种慢性自身免疫性疾病，其特征为血液学异常，包括贫血、白细胞减少和血小板减少。补体系统紊乱与SLE的发病机制有关。在本研究中，我们旨在探讨对补体系统进行全面评估（包括对其三条途径的评估）与SLE患者群体血细胞计数之间的关系。对284例SLE患者进行了补体系统经典途径、替代途径和凝集素途径的新一代功能检测。此外，还评估了无活性分子（C1q、C2、C3、C4、因子D）和活性分子（C3a）以及调节因子（C1抑制剂和因子H）的血清水平。分析了全血细胞计数。进行多变量线性回归分析以研究血液学特征与补体系统这种全面表征之间的关系。在进行包括年龄、性别、SLICC-DI（损伤）和SLEDAI（活动）评分以及使用阿司匹林、泼尼松、甲氨蝶呤、硫唑嘌呤和霉酚酸酯的多变量调整后，观察到补体途径与单个产物和血细胞特征之间存在多种关系。C1q和C2值较低与血红蛋白水平较低相关。白细胞计数较低者的C4、C1抑制剂、C3、因子D和替代途径功能水平显著较低。中性粒细胞计数仅与替代途径和C1-inh呈显著负相关。对于淋巴细胞，发现了相关性，特别是与经典途径和替代途径的功能测试以及与C2、C4、C3和C3a的相关性。相反，对于血小板，在多变量调整后，仅观察到C2浓度较低时有显著性。总之，在调整疾病活动和损伤后，SLE中的血清补体系统与血液学特征独立相关。这些关系基本为负相关，且主要见于淋巴细胞。
