Nephrology Division, University of São Paulo, School of Medicine, São Paulo, Brazil.
Lupus. 2012 Aug;21(9):978-83. doi: 10.1177/0961203312443421. Epub 2012 Mar 26.
To evaluate clinicopathological features and treatment response in patients with lupus nephritis (LN), comparing the childhood- and late-onset forms of the disease.
We retrospectively analyzed clinical presentation, treatment and evolution in patients diagnosed with LN by renal biopsy between 1999 and 2008. Patients were grouped by age-≤18 years (n = 23); and ≥50 years (n = 13)-and were followed for the first year of treatment.
The baseline features of the childhood- and late-onset groups, respectively, were as follows: mean age, 15 ± 2 and 54 ± 5 years; female gender, 87% and 92%; hypertension, 87% and 77%; Systemic Lupus Erythematosus Disease Activity Index, 29 ± 9 and 17 ± 7 (p = 0.002); estimated glomerular filtration rate (eGFR), 86 ± 66 and 70 ± 18 ml/min; concurrent SLE/LN diagnosis, 90% and 15% (p < 0.001); crescents on biopsy, 74% and 30% (p = 0.02); activity index on biopsy, 4.8 ± 2.6 and 3.3 ± 1.9 (p = 0.10); and interstitial fibrosis (>10%), 39% and 61% (p = 0.08). Treatment consisted mainly of methylprednisolone, prednisone and intravenous cyclophosphamide, average cumulative doses being similar between the groups. After 12 months of treatment, the eGFR in the younger and older patients was 116 ± 62 and 78 ± 20 ml/min, respectively (p = 0.005). Three of the younger patients progressed to dialysis at 12 months, compared with none of the older patients.
Childhood-onset LN seems to be more severe than is late-onset LN.
比较幼年起病和晚发狼疮肾炎(LN)患者的临床病理特征和治疗反应。
我们回顾性分析了 1999 年至 2008 年间经肾活检诊断为 LN 的患者的临床表现、治疗和转归。根据年龄将患者分为≤18 岁(n=23)和≥50 岁(n=13)两组,并在治疗的第 1 年进行随访。
幼年组和晚发组的基线特征分别为:平均年龄 15±2 岁和 54±5 岁;女性占 87%和 92%;高血压占 87%和 77%;系统性红斑狼疮疾病活动指数(SLEDAI)为 29±9 和 17±7(p=0.002);估算肾小球滤过率(eGFR)为 86±66 和 70±18ml/min;同时诊断为系统性红斑狼疮/狼疮肾炎(SLE/LN)占 90%和 15%(p<0.001);活检中新月体占 74%和 30%(p=0.02);活检时的活动指数为 4.8±2.6 和 3.3±1.9(p=0.10);间质纤维化(>10%)占 39%和 61%(p=0.08)。治疗主要采用甲泼尼龙、泼尼松和静脉环磷酰胺,两组的累积剂量相似。治疗 12 个月后,年轻患者和老年患者的 eGFR 分别为 116±62 和 78±20ml/min(p=0.005)。3 名年轻患者在 12 个月时进展为透析,而老年患者无一例进展。
幼年起病的 LN 似乎比晚发的 LN 更严重。