Longterm followup of childhood lupus nephritis

• Published in: Journal of rheumatology 2002-12, Vol.29 (12), p.2635-2642
• Main Authors: HAGELBERG, Stefan, LEE, Yuna, SILVERMAN, Earl, BARGMAN, Joanne, MAH, Gordon, SCHNEIDER, Rayfel, LASKIN, Carl, EDDY, Allison, GLADMAN, Dafna, UROWITZ, Murray, HEBERT, Diane
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age of Onset; Azathioprine - therapeutic use; Biological and medical sciences; Canada - epidemiology; Child; Child, Preschool; Cyclophosphamide - therapeutic use; Female; Follow-Up Studies; Humans; Immunosuppressive Agents - therapeutic use; Kidney Failure, Chronic - etiology; Kidney Failure, Chronic - mortality; Kidney Failure, Chronic - pathology; Lupus Nephritis - diagnosis; Lupus Nephritis - drug therapy; Lupus Nephritis - mortality; Male; Medical sciences; Medicin och hälsovetenskap; Prognosis; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Survival Analysis; Survival Rate; Treatment Outcome
• ISSN: 0315-162X; 1499-2752

OBJECTIVE: To determine the longterm outcome in children with onset of lupus nephritis before 18 years of age. METHODS: Sixty-seven patients with onset of lupus nephritis prior to age 18 were identified. The mean followup time was 11 years (range 5-19). The mean age at diagnosis was 13.2 years (range 4-17). The male:female ratio was 1:3.8. Renal biopsies were classified using the WHO classification. Fifteen patients had Class II, 8 patients Class III, 32 patients Class IV, and 11 patients Class V and one patient refused biopsy. The cohort consists of the 66 patients who had a renal biopsy. Five patients received cyclophosphamide (CYC) and 17 received azathioprine (AZA) as part of the initial treatment of Class IV nephritis. Eight additional patients received CYC because of a flare of disease while receiving AZA, and 8 other patients received AZA because of a flare of disease while taking prednisone therapy. RESULTS: Four patients died; 6 developed endstage renal disease (ESRD); all but one of the patients who died and/or had ESRD had WHO Class IV [diffuse proliferative glomerulonephritis (DPGN)]; only 2 Caucasians developed ESRD, although 16 out of 36 Caucasians had DPGN; serum creatinine at followup was normal in 84% of the survivors; presently 70% of the patients take less than 7.5 mg prednisone/day and 62% do not take cytotoxic drugs. No patient is currently treated with CYC. All 8 patients with Class III nephritis were taking medication at last followup. CONCLUSION: The longterm outcome in this group of children with lupus nephritis, in whom AZA was the most commonly used immunosuppressive agent, was excellent, with 94% patient survival at a mean followup of 11 years. Our results suggest that non-Caucasian patients with pediatric onset lupus nephritis may be at increased risk for renal failure compared to Caucasians.