Lupus Genomics and Global Disparities Unit, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Division of Nephrology, Boston Children's Hospital, Boston, MA, USA.
Lupus. 2021 Sep;30(10):1660-1670. doi: 10.1177/09612033211028658. Epub 2021 Jul 4.
Lupus nephritis (LN) is a life-threatening manifestation of systemic lupus erythematosus (SLE) and is more common in children than adults. The epidemiology and management of childhood-onset SLE (cSLE) have changed over time, prompting the need to reassess expected outcomes. The purpose of this study is to use the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry to validate historical principles of LN in a contemporary, real-world cohort. After an extensive literature review, six principles of LN in cSLE were identified. The CARRA registry was queried to evaluate these principles in determining the rate of LN in cSLE, median time from cSLE diagnosis to LN, short-term renal outcomes, and frequency of rituximab as an induction therapy. Of the 677 cSLE patients in the CARRA registry, 32% had documented LN. Decline in kidney function was more common in Black cSLE patients than non-Black patients ( = 0.04). Black race was associated with worse short-term renal outcomes. In short-term follow up, most children with LN had unchanged or improved kidney function, and end stage kidney disease (ESKD) was rare. Ongoing follow-up of cSLE patients in the CARRA registry will be necessary to evaluate long-term outcomes to inform risk, management, and prognosis of LN in cSLE.
狼疮性肾炎（LN）是系统性红斑狼疮（SLE）的一种危及生命的表现，在儿童中比在成人中更为常见。儿童发病的系统性红斑狼疮（cSLE）的流行病学和治疗方法随时间而变化，这促使我们需要重新评估预期结果。本研究的目的是使用儿童关节炎和风湿病研究联盟（CARRA）的前瞻性登记处，在当代真实世界队列中验证 LN 的历史原则。在广泛的文献回顾之后，确定了 cSLE 中 LN 的六个原则。对 CARRA 登记处进行了查询，以评估这些原则在确定 cSLE 中 LN 的发生率、从 cSLE 诊断到 LN 的中位时间、短期肾脏结局以及利妥昔单抗作为诱导治疗的频率方面的作用。在 CARRA 登记处的 677 例 cSLE 患者中，有 32%有记录的 LN。与非黑人患者相比，黑人 cSLE 患者的肾功能下降更为常见（ = 0.04）。黑种人种族与短期肾脏结局较差有关。在短期随访中，大多数患有 LN 的儿童的肾功能没有变化或有所改善，终末期肾病（ESKD）很少见。需要对 CARRA 登记处中的 cSLE 患者进行持续随访，以评估长期结局，从而为 cSLE 中的 LN 提供风险、管理和预后信息。
