Pediatric Rheumatology, Texas Children's Hospital, TX, Houston, USA.
Baylor College of Medicine, Houston, TX, USA.
Pediatr Nephrol. 2022 Dec;37(12):3139-3145. doi: 10.1007/s00467-022-05524-2. Epub 2022 Mar 28.
The revised 2018 ISN/RPS Classification System for lupus nephritis (LN) includes calculations for both activity index (A.I.) and chronicity index (C.I.). Unchanged were the thresholds of < 25%, 25-50%, and > 50% crescents to distinguish between mild, moderate, and severe activity/chronicity. We aimed to evaluate these thresholds for percent crescents in childhood-onset LN.
Eighty-six subjects < 21 years of age were enrolled from the Pediatric Glomerulonephritis with Crescents Registry, a retrospective multi-center cohort sponsored by the Pediatric Nephrology Research Consortium. Thresholds of 10%, 25%, and 50% for both cellular/fibrocellular and fibrous crescents were interrogated for primary outcomes of kidney failure, eGFR, and eGFR slope.
Median age at time of initial biopsy was 14 years (range 1-21). Median follow-up time was 3 years (range 1-11). Cumulative incidence of kidney failure was 6% at 1 year and 10% at latest follow-up. Median eGFR slope was - 18 mL/1.73 m/min (IQR - 51 to + 8) at 1 year and - 3 mL/min/1.73 m/year (IQR - 19 to + 6) at latest follow-up. We found no difference in kidney failure at the proposed < 25% and 25-50% cellular crescents thresholds, and thus added a new provisional threshold of 10% that better predicted outcomes in children. Moreover, use of 10% and 25% thresholds for fibrous crescents showed a fourfold and sevenfold increase in risk of kidney failure.
In children with crescentic LN, use of 10% and 25% thresholds for cellular crescents better reflects disease activity, while these thresholds for fibrous crescents better discriminates kidney disease outcomes. A higher resolution version of the Graphical abstract is available as Supplementary information.
修订后的 2018 年 ISN/RPS 狼疮肾炎（LN）分类系统包括活动指数（A.I.）和慢性指数（C.I.）的计算。不变的是，<25%、25-50%和>50%新月体的阈值用于区分轻度、中度和重度活动/慢性。我们旨在评估这些儿童发病的 LN 新月体百分比的阈值。
从儿科肾脏病研究联盟赞助的回顾性多中心队列小儿肾小球肾炎伴新月体登记处招募了 86 名年龄<21 岁的受试者。对 10%、25%和 50%的细胞/纤维细胞性和纤维性新月体进行了调查，主要结果为肾衰竭、eGFR 和 eGFR 斜率。
初次活检时的中位年龄为 14 岁（范围 1-21 岁）。中位随访时间为 3 年（范围 1-11 年）。1 年和最新随访时肾衰竭的累积发生率分别为 6%和 10%。1 年时 eGFR 斜率中位数为-18 mL/1.73 m/min（IQR-51 至+8），最新随访时为-3 mL/min/1.73 m/year（IQR-19 至+6）。我们发现，在提议的<25%和 25-50%细胞新月体阈值中，肾衰竭没有差异，因此增加了一个新的 10%的暂定阈值，该阈值能更好地预测儿童的结果。此外，使用 10%和 25%的纤维新月体阈值会使肾衰竭的风险增加 4 倍和 7 倍。
在患有新月体 LN 的儿童中，使用 10%和 25%的细胞新月体阈值能更好地反映疾病活动，而这些纤维新月体的阈值能更好地区分肾脏疾病的结果。一个更清晰的图表版本可在补充信息中查看。
