Pediatric Clinic, University of Brescia and ASST Spedali Civili di Brescia, Brescia, Italy.
Anna Meyer Children's Hospital and University of Florence, Viale Pieraccini 24, 50139, Florence, Italy.
Clin Rev Allergy Immunol. 2019 Jun;56(3):293-307. doi: 10.1007/s12016-017-8642-3.
Autoimmune diseases affect up to 10% of the world's population and, as a whole, they are far more common in females, although differences exist according to the single disease and also in different age groups. In childhood-onset autoimmune diseases, the sex bias is generally less evident than in adults, probably for the different hormonal milieau, being estrogens strongly implicated in the development of autoimmunity. Still, some rheumatic conditions, such as juvenile idiopathic arthritis (JIA), show a strong predilection for girls (F:M = 3-6.6:1), and differences may coexist between males and females regarding disease outcome. For example, chronic anterior uveitis associated with JIA affects more commonly girls but boys tend to have a more severe course. Systemic lupus erythematosus predominantly affects girls and women (F:M = 3-5:1 in children, F:M = 10-15:1 in adults). Behςet's disease has been reported to be more prevalent in adult males (F:M = 1:1-4); in children, there are no differences. The sex ratio is equal in children and adults for Henoch-Schönlein purpura (F:M = 1:1). A higher male-to-female ratio exists for Kawasaki disease (F:M = 1:1.1-1.6 in children, F:M = 1:1,5 in adults). Juvenile dermatomyositis (F:M = 2-5:1), systemic sclerosis (F:M = 4:1 in children, F:M = 6:1 in adults), and Takayasu arteritis (F:M = 2:1 in children, F:M = 7-9:1 in adults) are more common in girls and women then in boys and men. There is no gender bias for acute rheumatic fever in children, while in adults, the F:M ratio is 2:1. Given that estrogen levels are not different between genders during childhood, pediatric rheumatic diseases could represent good models to study other mechanisms related to the development of autoimmunity. Recently, the levels of miRNA expression, and their variation according to sex chromosomes, have been linked to the development of autoimmune diseases, with different impact among sexes. This review will focus not only on the sex bias reported in the more common rheumatic conditions of childhood, focusing on differences in incidence, but also on outcome and trying to depict the mechanisms underlying those differences.
自身免疫性疾病影响全球多达 10%的人口，总的来说，女性比男性更容易患此类疾病，尽管每种疾病以及不同年龄组之间存在差异。在儿童期发病的自身免疫性疾病中，性别差异通常不如成年人明显，这可能与不同的激素环境有关，雌激素强烈参与了自身免疫的发展。尽管如此，一些风湿性疾病，如幼年特发性关节炎（JIA），仍然对女孩有强烈的偏好（F:M=3-6.6:1），而且疾病结局在男性和女性之间可能存在差异。例如，与 JIA 相关的慢性前葡萄膜炎更常见于女孩，但男孩的病情往往更严重。系统性红斑狼疮主要影响女孩和妇女（儿童中 F:M=3-5:1，成人中 F:M=10-15:1）。贝切特病在成年男性中更为常见（F:M=1:1-4）；在儿童中，没有差异。儿童和成人的亨诺克-舍恩莱因紫癜的性别比例相等（F:M=1:1）。川崎病的男女比例较高（儿童中 F:M=1:1.1-1.6，成人中 F:M=1:1.5）。幼年型皮肌炎（F:M=2-5:1）、系统性硬化症（儿童中 F:M=4:1，成人中 F:M=6:1）和 Takayasu 动脉炎（儿童中 F:M=2:1，成人中 F:M=7-9:1）在女孩和妇女中比男孩和男子更为常见。儿童急性风湿热无性别偏见，而成人 F:M 比为 2:1。鉴于儿童期男女的雌激素水平没有差异，儿科风湿性疾病可能是研究与自身免疫发展相关的其他机制的良好模型。最近，miRNA 表达水平及其根据性染色体的变化与自身免疫性疾病的发展有关，在性别间的影响不同。本综述不仅将重点讨论儿童期更为常见的风湿性疾病中报告的性别差异，关注发病率的差异，还将关注结局，并试图描绘这些差异背后的机制。
