Adriana Suhlrie1,2, Imke Hennies1,2, Jutta Gellermann3, Anja Büscher4, Peter Hoyer4, Siegfried Waldegger5, Simone Wygoda6, Rolf Beetz7, Bärbel Lange-Sperandio8, Günter Klaus9, Martin Konrad10, Martin Holder11, Hagen Staude12, Wolfgang Rascher13, Jun Oh14, Lars Pape1,2, Burkhard Tönshoff15, Dieter Haffner16,17. 1. Department of Paediatric Kidney, Liver and Metabolic Diseases, Paediatric Research Center, Hannover Medical School Children's Hospital, Carl-Neuberg-Str. 1, 30625, Hannover, Germany. 2. Center for Rare Diseases, Hannover Medical School Children's Hospital, Hannover, Germany. 3. Department of Paediatrics, University Children's Hospital Berlin, University Hospital, Berlin Charité, Berlin, Germany. 4. Department of Paediatrics II, University Hospital Essen, Essen, Germany. 5. Department of Peadiatrics I, Medical University of Innsbruck, Innsbruck, Austria. 6. St. Georg Children's Hospital, Leipzig, Germany. 7. University Children's Hospital Mainz, Mainz, Germany. 8. Dr. v. Hauner Children's Hospital, Division of Paediatric Nephrology, Ludwig-Maximilians, University of Munich, Munich, Germany. 9. University Children's Hospital Marburg, Marburg, Germany. 10. Department of General Paediatrics, University Children's Hospital, Münster, Germany. 11. Department of Pediatrics, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany. 12. University Children's Hospital Rostock, Rostock, Germany. 13. Department of Paediatrics and Adolescent Medicine, University Hospital Erlangen, Erlangen, Germany. 14. Department of Paediatrics, University Hamburg-Eppendorf, 20246, Hamburg, Germany. 15. Department of Paediatrics I, University Children's Hospital Heidelberg, University of Heidelberg, Heidelberg, Germany. 16. Department of Paediatric Kidney, Liver and Metabolic Diseases, Paediatric Research Center, Hannover Medical School Children's Hospital, Carl-Neuberg-Str. 1, 30625, Hannover, Germany. Haffner.Dieter@mh-hannover.de. 17. Center for Rare Diseases, Hannover Medical School Children's Hospital, Hannover, Germany. Haffner.Dieter@mh-hannover.de.
Abstract
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.
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.
Authors: Kristina Vollbach; Catharina Schuetz; Christian M Hedrich; Fabian Speth; Kirsten Mönkemöller; Jürgen Brunner; Ulrich Neudorf; Christoph Rietschel; Anton Hospach; Tilmann Kallinich; Claas Hinze; Norbert Wagner; Burkhard Tönshoff; Lutz T Weber; Kay Latta; Julia Thumfart; Martin Bald; Dagobert Wiemann; Hildegard Zappel; Klaus Tenbrock; Dieter Haffner Journal: Front Pediatr Date: 2022-04-22 Impact factor: 3.569