Wegener-Granulomatose bei pädiatrischen Patienten

Zusammenfassung

Die Wegener-Granulomatose (WG) ist eine im Kindes- und Jugendalter selten auftretende Erkrankung. Sie gehört neben dem Churg-Strauss-Syndrom und der mikroskopischen Polyangiitis zu den ANCA assoziierten Vaskuli-tiden. Vornehmlich betrifft die WG den oberen sowie unteren Atemwegstrakt und die Nieren. Die Krankheit ist gekennzeichnet durch einen chronischen Verlauf mit hoher Rezidivneigung. Bei persistierenden Beschwerden im Bereich des Respirationstraktes, welche sich nicht als Infektion oder Allergie bestätigen, ist differenzialdiagnostisch auch an eine WG zu denken.Trotz gebesserter Prognose seit Einführung der immunsuppressiven Therapie bleibt die WG eine sehr ernst zu nehmende Erkrankung, welche in ca. einem Drittel der Fälle zu chronischer Niereninsuffizienz bis zum terminalen Nierenversagen führen kann. Frühe Kombinationsbehandlung mit dem Ziel der Remission und Verbesserung der Prognose ist dringend indiziert. Zur Erhaltungstherapie sollten aufgrund der Langfristigkeit der Behandlung weniger toxische Immunsuppressiva (z. B. Methotrexat) verwendet werden.

Abstract

Wegener's Granulomatosis (WG) is a disease occurring rarely in childhood and adolescence. Together with the Churg-Strauss-Syndrome and the microscopic Polyangiitis it belongs to the vasculitis syndromes associated with ANCA. WG mostly affects the upper and lower respiratory tract and kidneys. It is characterized by a chronic development and high tendency to relapse. In cases of persistent disturbances of the respiratory system which do not have infectious or allergic geneses differential diagnosis should also consider WG.Although clinical course and prognosis have improved since the introduction of immunosuppressive therapy, WG, leading to renal failure in about one third of all cases, remains a disease which has to be taken seriously. Early treatment has been proven to improve prognosis. Due to the necessity of longterm treatment less toxic immunosuppressive therapy should be applied (e.g. Methotrexate).

Schlüsselwörter

Wegener-Granulomatose - Vaskulitis - Vaskulitiden Therapie - Kindesalter

Key words

Wegener's granulomatosis - vasculitis - vasculitis treatment - childhood

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