Wenderfer Scott E, Eldin Karen W
Pediatric Nephrology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, 1102 Bates Avenue, Suite 245, Houston, TX 77030, USA.
Department of Pathology and Immunology, Baylor College of Medicine, Texas Children's Hospital, 6621 Fannin Street, Suite AB195, Houston, TX 77030, USA.
Pediatr Clin North Am. 2019 Feb;66(1):87-99. doi: 10.1016/j.pcl.2018.08.007.
Childhood-onset systemic lupus erythematosus (SLE) is a subset of SLE with an onset before 18 years of age. Patients with early onset SLE tend to have a greater genetic component to their disease cause, more multisystemic involvement, and a more severe disease course, which includes greater risks for developing nephritis and end-stage kidney disease. Five- and 10-year mortality is lower than in adult-onset SLE. Although patient and renal survival have improved with advances in induction and maintenance immunosuppression, accumulation of irreversible damage is common. Cardiovascular and infectious complications are frequent, as are relapses during adolescence and the transition to adulthood.
儿童期起病的系统性红斑狼疮(SLE)是SLE的一个亚组,发病年龄在18岁之前。早发性SLE患者的疾病病因往往有更大的遗传因素,多系统受累更多,疾病进程更严重,包括发生肾炎和终末期肾病的风险更高。5年和10年死亡率低于成人起病的SLE。尽管随着诱导和维持免疫抑制的进展,患者生存率和肾脏生存率有所提高,但不可逆损伤的积累很常见。心血管和感染并发症频繁,青春期及向成年期过渡期间的复发也很常见。