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Twelve-month outcome in juvenile proliferative lupus nephritis: results of the German registry study
Adriana Suhlrie1,2, Imke Hennies1,2, Jutta Gellermann3
1Department of Paediatric Kidney, Liver and Metabolic Diseases, Paediatric Research Center, Hannover Medical School Children's Hospital, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Insights
Pediatric lupus nephritis (LN) treatment shows good renal outcomes but high glucocorticoid toxicity. This highlights the need for reduced corticosteroid use in juvenile lupus nephritis management.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunosuppressive Therapy
Background:
- Children with proliferative lupus nephritis (LN) often receive intensive immunosuppressive treatments.
- Limited data exists on the long-term renal outcomes and treatment toxicity in this pediatric population.
Purpose of the Study:
- To assess the 12-month renal outcome and comorbidity in children with proliferative lupus nephritis.
- To evaluate the impact of treatment protocols on outcomes and identify associated complications.
Main Methods:
- Retrospective analysis of 79 predominantly Caucasian children with proliferative LN.
- Data collected from the Lupus Nephritis Registry of the German Society of Paediatric Nephrology (1997-2015).
- Assessment of renal function, proteinuria, and treatment-related toxicities at 12 months post-diagnosis.
Main Results:
- At 12 months, 38% achieved complete remission and 41% partial remission; 15% experienced renal flare.
- Nephrotic range proteinuria at diagnosis correlated with poorer renal outcomes (OR 5.34, P=.02).
- High rates of complications: 80% experienced adverse events, including 42% with glucocorticoid toxicity and 78% with growth impairment.
Conclusions:
- Juvenile proliferative lupus nephritis demonstrates favorable renal outcomes at 12 months, regardless of induction therapy (MMF vs. IVCYC).
- High incidence of glucocorticoid toxicity necessitates the development of corticosteroid-sparing treatment strategies.
- Further research is needed to optimize treatment protocols and minimize long-term adverse effects in pediatric LN.
Background:
Children presenting with proliferative lupus nephritis (LN) are treated with intensified immunosuppressive protocols. Data on renal outcome and treatment toxicity is scare.
Methods:
Twelve-month renal outcome and comorbidity were assessed in 79 predominantly Caucasian children with proliferative LN reported to the Lupus Nephritis Registry of the German Society of Paediatric Nephrology diagnosed between 1997 and 2015.
Results:
At the time of diagnosis, median age was 13.7 (interquartile range 11.8-15.8) years; 86% showed WHO histology class IV, nephrotic range proteinuria was noted in 55%, and median estimated glomerular filtration rate amounted to 75 ml/min/1.73 m2. At 12 months, the percentage of patients with complete and partial remission was 38% and 41%, respectively. Six percent of patients were non-responders and 15% presented with renal flare. Nephrotic range proteinuria at the time of diagnosis was associated with inferior renal outcome (odds ratio 5.34, 95% confidence interval 1.26-22.62, p = 0.02), whereas all other variables including mode of immune-suppressive treatment (e.g., induction treatment with cyclophosphamide (IVCYC) versus mycophenolate mofetil (MMF)) were not significant correlates. Complications were reported in 80% of patients including glucocorticoid toxicity in 42% (Cushingoid appearance, striae distensae, cataract, or osteonecrosis), leukopenia in 37%, infection in 23%, and menstrual disorder in 20%. Growth impairment, more pronounced in boys than girls, was noted in 78% of patients.
Conclusions:
In this cohort of juvenile proliferative LN, renal outcome at 12 months was good irrespectively if patients received induction treatment with MMF or IVCYC, but glucocorticoid toxicity was very high underscoring the need for corticoid sparing protocols. Graphical abstract.
