Lupus nephritis and systemic disease

Overview & Definition

A 13-year-old girl who has been losing weight and complaining of swollen fingers presents with puffy eyelids, dark urine, and a blood pressure of 135 over 85. She has oral ulcers and a faint rash across her cheeks. Her urine shows blood and protein, her creatinine is mildly elevated, and her complement C3 and C4 are low. This is lupus nephritis, the renal manifestation of systemic lupus erythematosus, in which autoantibodies form immune complexes that deposit in the glomerulus and trigger inflammation. It is one of the most serious complications of childhood-onset lupus and the single strongest predictor of long-term morbidity and mortality in the disease. [9]

Renal involvement is both more frequent and more severe in children than in adults. Up to 50 to 80 percent of children with systemic lupus erythematosus develop nephritis, compared with roughly 30 to 40 percent of adults, and children are more likely to present with the aggressive proliferative Class III and IV patterns. Most childhood-onset lupus is diagnosed in adolescence, with a striking female predominance driven by oestrogen, and disease onset before puberty tends to be more severe. Because the kidney can be the first or only obviously affected organ, any child with unexplained haematuria, proteinuria, hypertension, or declining renal function needs lupus excluded. [9]

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