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Arthritis und Rheuma 2014; 34(03): 169-174
DOI: 10.1055/s-0037-1617988
Kinderrheumatologie: Übersichtsartikel
Schattauer GmbH

Primäre Immundefekte und Autoimmunität

Rheumatologische Manifestationen bei pädiatrischen Patienten mit primären ImmundefektenPrimary immunodeficiency and autoimmunityRheumatological manifestations in pediatric patients with primary immunodeficiency
A. Meyer-Bahlburg
1   Klinik für pädiatrische Pneumologie, Allergologie und Neonatologie, Medizinische Hochschule Hannover
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Publication History

Publication Date:
27 December 2017 (online)

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Zusammenfassung

Primäre Immundefekte sind seltene Erkrankungen, die durch eine erhöhte Infektanfälligkeit und das Auftreten schwerer und opportunistischer Infektionen gekennzeichnet sind. Trotz des Immundefektes treten häufig Autoimmunsymptome auf. Dabei steht bei einigen Immundefekten die Autoimmunität im Vordergrund, wohingegen bei anderen Immundefekten Autoimmunsymptome eher als eine Komplikation angesehen werden. In Bezug auf rheumatologische Manifestationen treten insbesondere chronische Arthritiden, aber auch ein systemischer Lupus erythemodes oder Vaskulitiden auf. Die Pathogenese der Autoimmunität bei primären Immundefekten ist je nach Erkrankung unterschiedlich und nicht vollständig verstanden. Therapeutisch empfiehlt sich zunächst eine Behandlung gemäß den entsprechenden Richtlinien. Häufig gestaltet sich die Behandlung jedoch schwierig, so dass alternative Behandlungsstrategien versucht werden müssen. Bei vielen primären Immundefekten stellt inzwischen die Stammzelltransplantation einen kurativen Lösungsansatz dar. Diese führt bei erfolgreicher Durchführung meistens auch zur Remission der Autoimmunsymptome.

Summary

Primary immunodeficiencies are rare diseases. Patients have an increased susceptibility to infection and suffer from severe and opportunistic infections. Despite the immuno deficiency, patients often also suffer from autoimmune symptoms. Autoimmune symptoms can be the major characteristic of the primary immunodeficiency or only a complication. Regarding rheumatologic autoimmune manifestations, mainly chronic arthritis, systemic lupus erythematodes and vasculitis are observed. The pathogenesis of autoimmunity in primary immunodeficiency is diverse and not well understood. Rheumatologic symptoms should be treated according to the corresponding guidelines. However, treatment is often difficult and alternative therapies have to be considered. Stem cell transplantation offers a curative therapy for a variety of primary immunodeficiency and usually, if successful, also resolves autoimmune symptoms.

Schlüsselwörter

Primäre Immundefekte - Autoimmunität - chronische Arthritis - systemischer Lupus erythematodes

Keywords

Primary immunodeficiency - autoimmunity - chronic arthritis - systemic lupus erythematodes
 

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