Jhong-Yong Wu1, Kuo-Wei Yeh1, Jing-Long Huang2. 1. Department of Pediatrics, Division of Allergy, Asthma and Rheumatology, Chang Gung Memorial Hospital at Linkuo, Chang Gung University, 5, Fu-Hsin St, Taoyuan, Taiwan. 2. Department of Pediatrics, Division of Allergy, Asthma and Rheumatology, Chang Gung Memorial Hospital at Linkuo, Chang Gung University, 5, Fu-Hsin St, Taoyuan, Taiwan. Electronic address: long@adm.cgmh.org.tw.
Abstract
OBJECTIVES: Studies regarding the clinical features and outcomes of pediatric lupus nephritis are limited, and risk factors of poor outcome in the more severe form of renal involvement, proliferative lupus nephritis (PLN), are poorly understood. This study analyzed the data from a large prospective cohort of childhood systemic lupus erythematosus to determine such risk factors. METHODS: Subjects younger than 18 years at onset with biopsy-proven lupus nephritis were enrolled. Data on baseline presentations and laboratory values, response to treatment, and outcomes were collected. Primary outcome measures were death or end-stage renal disease (ESRD). Survival analysis was done and predictors were approached. RESULTS: One hundred four patients with mean age of 12.4 ± 2.5 years (range, 4.0-17.2 years) and the female-to-male ratio of 5.94:1 were included. Among them, 81 patients had PLN and 23 had non-proliferative lupus nephritis. Those with PLN had significantly lower GFR, more proteinuria, more urine sediment, more hypertension, and poor early response to treatment (within 6 months). There was no significant difference in extra-renal manifestations. All poor outcomes happened in the PLN group. The prognostic factors were high baseline SLEDAI-2k >20 (HR, 6.76; p = 0.002), baseline GFR <60ml/min/m² (HR, 3.88; p = 0.022), and early responder (HR, 0.19; p = 0.013). CONCLUSIONS: Patients with pediatric lupus nephritis and high risk factor can be identified early by concomitantly considering their baseline features and early response, which provides a novel hint in decision for management decisions.
OBJECTIVES: Studies regarding the clinical features and outcomes of pediatric lupus nephritis are limited, and risk factors of poor outcome in the more severe form of renal involvement, proliferative lupus nephritis (PLN), are poorly understood. This study analyzed the data from a large prospective cohort of childhood systemic lupus erythematosus to determine such risk factors. METHODS: Subjects younger than 18 years at onset with biopsy-proven lupus nephritis were enrolled. Data on baseline presentations and laboratory values, response to treatment, and outcomes were collected. Primary outcome measures were death or end-stage renal disease (ESRD). Survival analysis was done and predictors were approached. RESULTS: One hundred four patients with mean age of 12.4 ± 2.5 years (range, 4.0-17.2 years) and the female-to-male ratio of 5.94:1 were included. Among them, 81 patients had PLN and 23 had non-proliferative lupus nephritis. Those with PLN had significantly lower GFR, more proteinuria, more urine sediment, more hypertension, and poor early response to treatment (within 6 months). There was no significant difference in extra-renal manifestations. All poor outcomes happened in the PLN group. The prognostic factors were high baseline SLEDAI-2k >20 (HR, 6.76; p = 0.002), baseline GFR <60ml/min/m² (HR, 3.88; p = 0.022), and early responder (HR, 0.19; p = 0.013). CONCLUSIONS:Patients with pediatric lupus nephritis and high risk factor can be identified early by concomitantly considering their baseline features and early response, which provides a novel hint in decision for management decisions.
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