Division of Rheumatology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MC 4010, Cincinnati, OH 45229, USA.
Rheum Dis Clin North Am. 2010 Feb;36(1):53-80, vii-viii. doi: 10.1016/j.rdc.2009.12.012.
Some complement deficiencies predispose to systemic lupus erythematosus (SLE) early in life. Currently, there are no known unique physiologic or genetic pathways that can explain the variability in disease phenotypes. Children present with more acute illness and have more frequent renal, hematologic, and central nervous system involvement compared to adults with SLE. Almost all children require corticosteroids during the course of their disease; many are treated with immunosuppressive drugs. Mortality rates remain higher with pediatric SLE. Children and adolescents accrue more damage, especially in the renal, ocular and musculoskeletal organ systems. Conversely, cardiovascular mortality is more prevalent in adults with SLE.
某些补体缺陷可导致红斑狼疮(SLE)在生命早期发生。目前,尚无已知的独特生理或遗传途径可以解释疾病表型的可变性。与成人 SLE 相比,儿童表现出更严重的疾病,并且肾脏、血液系统和中枢神经系统受累更频繁。几乎所有儿童在疾病过程中都需要皮质类固醇;许多人接受免疫抑制剂治疗。儿科红斑狼疮的死亡率仍然较高。儿童和青少年会累积更多的损伤,尤其是在肾脏、眼部和肌肉骨骼系统。相反,心血管疾病的死亡率在成人 SLE 中更为常见。