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Behandlung der Purpura-Schönlein-Henoch-Nephritis bei Kindern und Jugendlichen

Therapieempfehlungen der Gesellschaft für Pädiatrische Nephrologie (GPN)

Treatment of Henoch-Schönlein purpura nephritis in children and adolescents

Therapy recommendations of the German Society for Pediatric Nephrology (GPN)

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Zusammenfassung

Nierenbeteiligung bei Purpura Schönlein-Henoch

Bei der Purpura Schönlein-Henoch findet sich häufig eine Nierenbeteiligung, die in ausgeprägten Fällen zu einer chronischen Niereninsuffizienz führen und eine dauerhafte Nierenersatztherapie notwendig machen kann.

Datenlage

Bisher publizierte Erfahrungen und Studien geben keinen Aufschluss darüber, ab welchem klinischen und histologischen Schweregrad der Glomerulonephritis, zu welchem Zeitpunkt, mit welchen Medikamenten und wie aggressiv die Nierenbeteiligung behandelt werden muss. Weder bei Kindern noch bei Erwachsenen wurden bisher Studien durchgeführt, die Antworten auf diese Fragen geben. Bei Erwachsenen ist die Inzidenz der Purpura-Schönlein-Henoch-Nephritis (PSHN) deutlich niedriger als im Kindesalter, somit sind für diese Altersgruppe keine aussagekräftigen Studien zu erwarten. Auch im Kindes- und Jugendalter tritt die PSHN so selten auf, dass kontrollierte Therapiestudien nur mit sehr hohem Aufwand multizentrisch und international durchführbar wären.

Therapie

Angesichts des Fehlens evidenzbasierter Therapieempfehlungen werden aktuell bei erkrankten Kindern und Jugendlichen unterschiedliche Ansätze für die Behandlung einer PSHN verfolgt. Eine konsentierte therapeutische Strategie existiert bisher nicht. Die aktuell angewendeten und in dieser Konsensusarbeit empfohlenen Medikamente kommen in dieser Indikation meist „off label“ zum Einsatz. Da die Verantwortung für die Medikation und ihre Nebenwirkungen beim behandelnden Arzt liegt, sollte ein „off label use“ von Medikamenten gemäß dem derzeitigen wissenschaftlichen und klinischen Kenntnisstand erfolgen.

Schlussfolgerung

In der vorliegenden Konsensusempfehlung werden der aktuelle Stand der Literatur zur Therapie der PSHN dargelegt und eine Behandlungsstrategie unter Einschluss der Expertenerfahrung der Autoren abgeleitet. Durch die Vereinheitlichung der Therapie und die Dokumentation des Behandlungsverlaufs wird angestrebt, mehr Erfahrung über den Verlauf und das Therapieansprechen der PSHN zu gewinnen. Auf Basis dieser Daten wäre der Effekt künftiger Therapiemodifikationen besser beurteilbar. Voraussetzung hierfür sind die Erfassung und Verlaufsbeobachtung möglichst aller Patienten mit der Diagnose PSHN in dem hierfür etablierten GPN-Register (GPN: Gesellschaft für Pädiatrische Nephrologie).

Abstract

Renal involvement in Henoch-Schönlein purpura

Henoch-Schönlein purpura frequently affects the kidneys and can lead to chronic renal insufficiency or end-stage renal disease in severe cases.

Background

As appropriate controlled studies have not been conducted in either children or adults it has not been defined which cases of Henoch-Schönlein purpura nephritis (HSPN) should be treated, when they should be treated and which medication should be selected. In adults the incidence of HSPN is considerably lower than in children and treatment evidence is unlikely to be gained in adults. Even in children the incidence of severe HSPN is low so that randomized controlled treatment studies would require an international multicenter approach.

Therapy

As there is no consensus regarding the treatment of pediatric HSPN various protocols are used. The different treatment protocols for HSPN as well as the medication recommended in this consensus represent an off label use. The responsibility for the effects and side effects of the medication lies with the prescribing doctor and the treatment should be chosen based on current clinical and scientific knowledge.

Conclusions

The presented consensus evaluates the current literature and derives recommendations for the treatment of HSPN which reflects the expert opinion of the authors. Standardizing the therapy and documentation of the outcome aim at increasing experience with this entity and will allow better evaluation. To this end treatment modalities and long-term outcome of pediatric patients with HSPN are documented in a registry of the Society for Pediatric Nephrology (GPN).

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Abbreviations

ACE:

„Angiotensin converting enzyme“

AT1:

Angiotensin-II-Rezeptor Subtyp 1

AZA:

Azathioprin

C3:

Komplementfaktor 3

CPA:

Cyclophosphamid

CsA:

Ciclosporin A

ED:

Einzeldosis

GFR:

Glomeruläre Filtrationsrate

GnRH:

Gonadotropin-Releasing-Hormon

GPN:

Gesellschaft für Pädiatrische Nephrologie

IgA:

Immunglobulin A

IgAN:

IgA-Nephritis

IgG:

Immunglobulin G

IgM:

Immunglobulin M

ISKDC:

„International study of kidney disease in children“

KG:

Körpergewicht

Mesna:

2-Mercaptoethansulfonat-Natrium

MMF:

Mykophenolat Mofetil

PSH:

Purpura Schönlein-Henoch

PSHN:

Purpura-Schönlein-Henoch-Nephritis

TMP:

Trimetoprim

SMX:

Sulfamethoxazol

ZNS:

Zentralnervensystem

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  1. M. Pohl
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  2. K. Dittrich
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Gesellschaft für Pädiatrische Nephrologie (GPN)., Pohl, M., Dittrich, K. et al. Behandlung der Purpura-Schönlein-Henoch-Nephritis bei Kindern und Jugendlichen . Monatsschr Kinderheilkd 161, 543–553 (2013). https://doi.org/10.1007/s00112-013-2896-5

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