Lupus Nephritis in Children Renal involvement in SLE: 30% - 70% Renal involvement in SLE: 30% - 70% Most diagnosis in adolescence, rare < 5y/o Most diagnosis in adolescence, rare < 5y/o Classification of lupus nephritis: WHO classification :Table 1 Classification of lupus nephritis: WHO classification :Table 1 S/S: microscopic hematuria; proteinuria : mild to nephrotic range; hypertension; renal insufficiency S/S: microscopic hematuria; proteinuria : mild to nephrotic range; hypertension; renal insufficiency

Histological classification of lupus nephritis(1) Active index: cellular or fibrocellular crescents, endocapillary proliferation, fibrinoid necrosis, karyorrhexis, hyaline thrombi, wire loops with subendothelial immune deposits, glomerular leukocyte infiltration, and interstitial mononuclear cell infiltrational Active index: cellular or fibrocellular crescents, endocapillary proliferation, fibrinoid necrosis, karyorrhexis, hyaline thrombi, wire loops with subendothelial immune deposits, glomerular leukocyte infiltration, and interstitial mononuclear cell infiltrational Active lesion: each grade 0-3 to give an activity index grade 0-24 Active lesion: each grade 0-3 to give an activity index grade 0-24

Histological classification of lupus nephritis(2) Chronicity Index: glomerular sclerosis, fibrous crescents, tubular fibrosis, and interstitial fibrosis Chronicity Index: glomerular sclerosis, fibrous crescents, tubular fibrosis, and interstitial fibrosis These lesion did not response to treatment. These lesion did not response to treatment. Activity index > 12/24 or elevated chronicity index  poor renal prognosis Activity index > 12/24 or elevated chronicity index  poor renal prognosis 10- year renal survvial rate: 10- year renal survvial rate: Chronicity index: <2  100% Chronicity index: <2  100% 2~4  70% 2~4  70% > 4  35% > 4  35% ESRD incidence : 20 % ESRD incidence : 20 %

Therapy according to classification(1) Class I: normal. Class I: normal. Class II: mesangial GN. Need only follow up. Class II: mesangial GN. Need only follow up. Class III: Focal and segmental GN: Class III: Focal and segmental GN: –Without nephrotic sx, therapy is only indicate for extra-renal disease. –With nephrotic sx, aggressive therapy is needed as class IV disease.

Therapy according to classification(2) Class V: membranous nephropathy: Class V: membranous nephropathy:  Those with pure membranous nephropathy: good prognosis, need no therapy.  With nephrotic syndrome: with risk of thrombotic events.  With membranous nephropathy and proliferative lesions got the worst prognosis and should treated as class IV.

Treatment of Lupus Nephritis(3) Cyclophosphamide pulse: Cyclophosphamide pulse: 750 mg/m 2 as starting dose 750 mg/m 2 as starting dose 1000 mg/m 2 ; if WBC> 3,000/mm mg/m 2 ; if WBC> 3,000/mm 3 Given as monthly boluses for 6 months then every 3 months for 2 additional years Given as monthly boluses for 6 months then every 3 months for 2 additional years Hemorrhagic cystitis, nausea, vomiting transient alopecia and gonadal toxicity Hemorrhagic cystitis, nausea, vomiting transient alopecia and gonadal toxicity Accumulative dosage > 200mg/Kg  toxicity Accumulative dosage > 200mg/Kg  toxicity