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Therapieempfehlung zur Lupusnephritis bei Kindern und Jugendlichen

Treatment recommendations in children and adolescents with lupus nephritis

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Zusammenfassung

Die Nierenbeteiligung spielt unter den Organmanifestationen des systemischen Lupus erythematodes (SLE) eine entscheidende Rolle für die Morbidität und Mortalität. Die frühzeitige Diagnosestellung und adäquate Therapie der Lupusnephritis (LN) ist daher von großer Bedeutung. Im Folgenden wird anhand des derzeitigen Standes der Literatur und der Erfahrungen der Mitglieder der SLE-Arbeitsgruppe der Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN) eine Konsensusempfehlung zur Therapie der LN im Kindes- und Jugendalter dargelegt. In Abhängigkeit von der Schwere der initialen Organbeteiligung (Grad der Proteinurie, Nierenhistologie) und dem initialen Therapieansprechen werden neben Prednisolon ggf. zusätzlich Mycophenolat Mofetil, Azathioprin, Cyclophosphamid oder Cyclosporin A eingesetzt. Bei der Begleittherapie ist neben einer konsequenten antihypertensiven und nephroprotekiven Behandlung auch der Vermeidung möglicher Nebenwirkungen wie Gonadentoxizität und Wachstumsstörung Rechnung zu tragen. Eine zukünftige prospektive Erfassung der nach dieser Konsensusempfehlung behandelten LN-Patienten kann unseres Erachtens zur Erstellung von Therapierichtlinien für dieses Krankheitsbild beitragen.

Abstract

Among the organ manifestations of systemic lupus erythematosus (SLE), involvement of the kidneys plays a key role in morbidity and mortality. Early diagnosis and appropriate treatment of lupus nephritis (LN) is therefore very important. On the basis of the current literature and the experience gathered by members of the SLE working group of the German Society for Pediatric Nephrology (APN), the following article presents consensus recommendations for treating LN in children and adolescents. Depending on the severity of the initial organ involvement (extent of proteinuria, kidney histology) and the initial response to treatment, prednisolone is supplemented, where appropriate, by mycophenolate mofetil, azathioprine, cyclophosphamide or cyclosporine A. In addition to consistent antihypertensive and nephroprotective treatment, adjuvant therapy should attempt to avoid possible side effects such as gonad toxicity and growth disorder. We believe that future prospective recording of the LN patients treated in line with these consensus recommendations can help treatment guidelines to be drawn up for this disease.

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Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN). Therapieempfehlung zur Lupusnephritis bei Kindern und Jugendlichen. Monatsschr Kinderheilkd 155, 1175–1188 (2007). https://doi.org/10.1007/s00112-007-1634-2

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