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Longterm followup of childhood lupus nephritis
Stefan Hagelberg1, Yuna Lee, Joanne Bargman
1Divisions of Rheumatology and Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Pediatric lupus nephritis shows excellent long-term outcomes with 94% survival, primarily treated with azathioprine (AZA). Non-Caucasian children may face a higher risk of renal failure in pediatric lupus nephritis.
Area of Science:
- Pediatric Nephrology
- Autoimmune Diseases
- Immunology
Background:
- Pediatric lupus nephritis, a severe manifestation of childhood-onset systemic lupus erythematosus, requires effective long-term management strategies.
- Understanding the long-term prognosis is crucial for optimizing treatment and improving patient outcomes.
Purpose of the Study:
- To evaluate the long-term outcomes of lupus nephritis in children diagnosed before 18 years of age.
- To identify factors influencing renal survival and overall patient prognosis.
Main Methods:
- Retrospective analysis of 67 children with pediatric-onset lupus nephritis.
- Mean follow-up of 11 years, with renal biopsies classified by WHO criteria.
- Treatment regimens included cyclophosphamide (CYC) and azathioprine (AZA); detailed treatment data were collected.
Main Results:
- Excellent patient survival at 94% over an 11-year follow-up period.
- End-stage renal disease (ESRD) and mortality were predominantly associated with WHO Class IV diffuse proliferative glomerulonephritis (DPGN).
- Serum creatinine normalized in 84% of survivors; most patients were on low-dose prednisone and not on cytotoxic drugs at follow-up.
Conclusions:
- Pediatric-onset lupus nephritis demonstrates favorable long-term outcomes, particularly when treated with azathioprine (AZA).
- Non-Caucasian children with pediatric lupus nephritis may have an elevated risk of renal failure compared to Caucasian counterparts.
- Aggressive immunosuppressive therapy, guided by renal biopsy, is essential for managing pediatric lupus nephritis.
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.