Working Towards a Treat-to-Target Protocol in Juvenile Proliferative Lupus Nephritis - A Survey of Pediatric Rheumatologists and Nephrologists in Germany and Austria

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

doi:10.3389/fped.2022.851998

Working Towards a Treat-to-Target Protocol in Juvenile Proliferative Lupus Nephritis - A Survey of Pediatric Rheumatologists and Nephrologists in Germany and Austria

Front Pediatr. 2022 Apr 22:10:851998. doi: 10.3389/fped.2022.851998. eCollection 2022.

Authors

Kristina Vollbach¹, Catharina Schuetz², Christian M Hedrich^{3

4}, Fabian Speth⁵, Kirsten Mönkemöller⁶, Jürgen Brunner⁷, Ulrich Neudorf⁸, Christoph Rietschel⁹, Anton Hospach¹⁰, Tilmann Kallinich¹¹, Claas Hinze¹², Norbert Wagner¹, Burkhard Tönshoff¹³, Lutz T Weber¹⁴, Kay Latta¹⁵, Julia Thumfart¹⁶, Martin Bald¹⁷, Dagobert Wiemann¹⁸, Hildegard Zappel¹⁹, Klaus Tenbrock¹, Dieter Haffner²⁰

Affiliations

¹ Department of Pediatrics, RWTH Aachen University Hospital, Aachen, Germany.
² Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
³ Department of Pediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United Kingdom.
⁴ Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom.
⁵ Universitätsmedizin Hamburg, Kinder- und Jugendklinik, Hamburg, Germany.
⁶ Department of Pediatrics, Kinderkrankenhaus Amsterdamer Strasse, Cologne, Germany.
⁷ Department of Pediatrics, Pediatric Rheumatology, Medizinische Universität Innsbruck, Innsbruck, and Danube Private University, Krems an der Donau, Austria.
⁸ Clinic for Pediatrics III, University Hospital Essen, Essen, Germany.
⁹ Department of Pediatric Rheumatology, Clementine Kinderhospital, Frankfurt, Germany.
¹⁰ Center for Pediatric Rheumatology, Olgahospital, Stuttgart, Germany.
¹¹ German Rheumatism Research Center, Leibniz Institute, Berlin, and Charité Universitätsmedizin Berlin, Pediatric Pneumology, Immunology and Critical Care Medicine and SPZ (Center for Chronically Sick Children), Berlin, Germany.
¹² Department of Pediatric Rheumatology and Immunology, University Hospital, Münster, Germany.
¹³ Department of Pediatrics I, University Children's Hospital, Heidelberg, Germany.
¹⁴ Division of Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Faculty of Medicine, University of Cologne, Cologne, Germany.
¹⁵ Clementine Kinderhospital Frankfurt, Frankfurt, Germany.
¹⁶ Department of Pediatric Gastroenterology, Nephrology and Metabolic Diseases, Charité Universitätsmedizin Berlin, Berlin, Germany.
¹⁷ Division of Pediatric Nephrology, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
¹⁸ Division of Pediatric Diabetology/Endocrinology, University Hospital Magdeburg, Magdeburg, Germany.
¹⁹ Clinic of Pediatrics and Adolescent Medicine, University Medical Center Göttingen, Göttingen, Germany.
²⁰ Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hanover, Germany.

Abstract

Background: To describe treatment practices for juvenile proliferative lupus nephritis (LN) class III and IV of pediatric rheumatologists and nephrologists in Germany and Austria in preparation for a treat-to-target treatment protocol in LN.

Methods: Survey study by members of the Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Pediatric Nephrology (GPN) on diagnostics and (concomitant) therapy of LN.

Results: Fifty-eight physicians completed the survey. Overall, there was a considerable heterogeneity regarding the suggested diagnostics and management of juvenile proliferative LN. Increased urinary protein excretion, either assessed by 24 h urine collection or spot urine (protein-creatinine ratio), and reduced estimated glomerular filtration rate were specified as important parameters for indication of kidney biopsy to diagnose proliferative LN and monitoring of therapy. Corticosteroids were generally proposed for induction and maintenance therapy, most often in conjunction with either mycophenolate mofetil (MMF) or cyclophosphamide (CP) as steroid-sparing immunosuppressants. MMF was clearly preferred over CP for induction therapy of LN class III, whereas CP and MMF were equally proposed for LN class IV. MMF was most often recommended for maintenance therapy in conjunction with oral corticosteroids and continued for at least 3 years and 1 year, respectively, after remission. Hydroxychloroquine was widely accepted as a concomitant measure followed by renin-angiotensin system inhibitors in cases of arterial hypertension and/or proteinuria.

Conclusion: The majority of pediatric rheumatologists and nephrologists in Germany and Austria propose the use of corticosteroids, most often in combination with either MMF or CP, for treatment of proliferative LN in children. The considerable heterogeneity of responses supports the need for a treat-to-target protocol for juvenile proliferative LN between pediatric rheumatologists and nephrologists.

Keywords: SLE; T2T; corticosteroid; cyclophosphamide; kidney biopsy; mycophenolate mofetil; nephritis.

Copyright © 2022 Vollbach, Schuetz, Hedrich, Speth, Mönkemöller, Brunner, Neudorf, Rietschel, Hospach, Kallinich, Hinze, Wagner, Tönshoff, Weber, Latta, Thumfart, Bald, Wiemann, Zappel, Tenbrock and Haffner.