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Vaskulitiden im Kindesalter

Childhood vasculitis

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Zusammenfassung

Hintergrund

Primäre Vaskulitiden im Kindesalter sind sehr selten. Die häufigsten Formen sind die Purpura Schönlein-Henoch und das Kawasaki-Syndrom, welche einen selbstlimitierenden Verlauf zeigen. Die Ätiopathogenese der Vaskulitiden ist zumeist noch unklar. Umweltfaktoren wie Infektionen scheinen bei prädisponierten Personen eine verstärkte inflammatorische Antwort auszulösen.

Ziel

In dem vorliegenden Artikel werden die verschiedenen Aspekte der Vaskulitiden im Kindesalter beschrieben.

Material und Methoden

Für die Zusammenstellung wurden die verfügbaren Übersichtsarbeiten und herausgehobene Originalarbeiten zu Vaskulitiden im Kindesalter, die vorliegenden Klassifikationskriterien und die aktuellen Therapieempfehlungen gesichtet und zusammengefasst.

Ergebnisse

Im Jahr 2008 ergänzte die europäische Fachgesellschaft für Kinderrheumatologie (PRES) die Klassifikationskriterien der Vaskulitiden im Erwachsenenalter um die Vaskulitiden im Kindesalter. Die meisten Therapieempfehlungen leiten sich jedoch aus Studienergebnissen aus dem Erwachsenenalter ab. Diese Übersichtsarbeit fasst die aktuellen Klassifikationskriterien, Pathogenesen, klinischen Manifestationen und Therapieprotokolle bei Vaskulitiden im Kindesalter zusammen.

Diskussion

Auch wenn die Ätiopathogenese vieler Vaskulitiden im Kindesalter nach wie vor ungeklärt ist, stehen doch zielführende diagnostische Verfahren und effektive therapeutische Optionen zur Verfügung. Die Kenntnis der unterschiedlichen Manifestationsformen kann zu einer frühen Diagnose und zu einer rechtzeitigen wirksamen Behandlung beitragen, die wiederum hilft, verheerende irreversible Langzeitschäden zu vermeiden.

Abstract

Background

The primary vasculitides are rare conditions in childhood. The most common disease subtypes are Schönlein-Henoch purpura and Kawasaki’s syndrome, which frequently have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental exposure, including infections, is suspected to trigger an autoinflammatory response in predisposed individuals.

Goal

The aim of this review is to present the various aspects of childhood vasculitis.

Materials and methods

Reviews and special original papers on childhood vasculitis, published classification criteria and current therapy guidelines were reviewed and summarized.

Results

The classification of vasculitides in childhood has been modified from the previous adult Chapel Hill classification for vasculitides in 2008. Most therapy recommendations for children are adapted from results of studies in adults. This review covers the current classifications, pathogenesis, clinical manifestations and therapy recommendations for children.

Discussion

Although etiology and pathogenesis of many vasculitides in childhood are still unknown, clarifying diagnostic methods and effective therapeutic options are available. The knowledge about various forms of disease manifestation may contribute to an early diagnosis and timely initiation of treatment, which may prevent devastating irreversible impairment.

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Abbreviations

AAV:

ANCA-assoziierte Vaskulitis

ACE:

Angiotensin Converting Enzyme

ACR:

American College of Rheumatology

ANCA:

Anti-Neutrophile zytoplasmatische Antikörper

ASS:

Acetylsalicylsäure

AT-1:

Angiotensin-1

BCG:

Bacillus Calmette-Guérin (Tuberkulose-Schutzimpfung)

BSG:

Blutkörperchensenkungsgeschwindigkeit

CHCC:

Chapel Hill Consensus Conference

cPAN:

Kutane Polyarteriitis nodosa

CRP:

C-reaktives Protein

CSS:

Churg-Strauss-Syndrom

CT:

Computertomographie

DD:

Differentialdiagnose

ECP:

Eosinophiles kationisches Protein

ED:

Einzeldosis

EEG:

Elektroenzephalographie

EGPA:

Eosinophile Granulomatose mit Polyangiitis

ELISA:

Enzyme-linked Immunosorbent Assay

EULAR:

European League Against Rheumatism

FMF:

Familiäres Mittelmeerfieber

γGT:

Gamma-Glutamyltransferase

GBM:

Glomeruläre Basalmembran

GFR:

Glomeruläre Filtrationsrate

GPA:

Granulomatose mit Polyangiitis

HDL:

High density lipoprotein

HNO:

Hals-Nasen-Ohren-Heilkunde

IL:

Interleukin

IVIG:

intravenöse Immunglobuline

KG:

Körpergewicht

KS:

Kawasaki-Syndrom

MESNA:

2-Mercaptoethansulfonat-Natrium

MPA:

Mikroskopische Polyangiitis

MRA:

Magnetresonanzangiographie

MRT:

Magnetresonanztomographie

MTX:

Methotrexat

NSAR:

Non steroidal anti-rheumatic drug

PAN:

Polyarteriitis nodosa

PET:

Positronenemissionstomographie

PNS:

Peripheres Nervensystem

PRES:

Paediatric Rheumatology European Society

PRINTO:

Paediatric Rheumatology International Trials Organisation

PSH:

Purpura Schönlein-Henoch

PVAS:

Modified Burmingham Vasculitis Activity Score

RR:

Riva-Rocci (nichtinvasive Blutdruckmessung)

sPAN:

systemische Polyarteriitis nodosa

TA:

Takayasu-Arteriitis

TNF:

Tumor Nekrose Faktor

WG:

Wegener-Granulomatose

ZNS:

Zentrales Nervensystem

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Authors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Autoinflammation Reference Center Tübingen, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 1, 72076, Tübingen, Deutschland

    J. B. Kümmerle-Deschner & J. Thomas

  2. Rheumatology, Department of Pediatrics, Alberta Children’s Hospital, University of Calgary, 2888 Shaganappi Trail NW, T3B 6A8, Calgary, Alberta, Canada

    S. M. Benseler

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  1. J. B. Kümmerle-Deschner
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  2. J. Thomas
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Correspondence to J. B. Kümmerle-Deschner.

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J. B. Kümmerle-Deschner, J. Thomas und S. M. Benseler geben an, dass kein Interessenkonflikt besteht.

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B. Hellmich, Kirchheim-Teck

P. Lamprecht, Lübeck

F. Moosig, Neumünster

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Kümmerle-Deschner, J.B., Thomas, J. & Benseler, S.M. Vaskulitiden im Kindesalter. Z Rheumatol 74, 863–877 (2015). https://doi.org/10.1007/s00393-015-0014-x

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