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Arthritis und Rheuma 2013; 33(04): 235-243
DOI: 10.1055/s-0037-1618189
Seltene Erkrankungen in der Rheumatologie
Schattauer GmbH

Systemische Sklerose

Eine schwere komplexe Erkrankung mit vielfältigen Komplikationen als Beispiel für Betreuung in der spezialärztlichen VersorgungSystemic sclerosisA severe multifaceted disease with variable complications and need for specialised care
K. Ahmadi-Simab
1   Asklepios Rheumazentrum Hamburg, Klinik für Rheumatologie, klinische Immunologie, Nephrologie, Asklepios Klinik Altona, Hamburg
,
G. Riemekasten
2   Rheumatologie und Klinische Immunologie, Charité Universitätsmedizin Berlin
› Institutsangaben
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Publikationsverlauf

Publikationsdatum:
19. Dezember 2017 (online)

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Zusammenfassung

Die systemische Sklerose (SSc, Sklerodermie) ist eine Erkrankung der Gefäße und des Bindegewebes mit hoher Relevanz für die Lebensqualität und Prognose. Die Seltenheit der Erkrankung, aber auch ihre Schwere, machen ein weiteres Erforschen der Pathogenese der Erkrankung dringend notwendig, so dass die Einbindung in Netzwerke und Forschungsverbünde empfohlen wird. Klinische Ausprägung und Verlauf der SSc zeigen eine große Variabilität mit Komplikationen in fast allen Organen, die ein interdisziplinäres Vorgehen erfordern. Das geringe Ansprechen auf Glukokortikoide und auf Immunsuppressiva sowie die obliterativen, verengenden Gefäßveränderungen grenzen diese Erkrankung von anderen Kollagenosen ab. Erhöhte Prävalenzen von Komorbiditäten wie Osteoporose, Krebsleiden, von Depressionen, von atherosklerotischen Gefäßveränderungen und einer Mangelernährung sind weitere Faktoren, die zur reduzierten Lebenserwartung und Lebensqualität beitragen. Die systemische Sklerose ist deshalb eine Erkrankung, die den Ansprüchen an eine Erkrankung mit der Betreuung über den § 116 b gerecht wird. In vorliegendem Artikel wird auf die vielfältigen Aspekte eingegangen, die bei der Betreuung dieser Patienten im Rahmen von Spezialambulanzen beachtet werden sollten.

Summary

Systemic sclerosis (SSc) is an inflammatory disease affecting small vessels and connective tissue, associated with poor prognosis, considerable burden of disease and reduced quality of life. Because of the fact that it is a severe and a rare disease there is urgent necessity to undertake more research on its pathogenesis and to coordinate research activities in networks. There is a great variability concerning clinical features and course of the disease, and multiorgan involvement demands interdisciplinary approach. Poor response to administration of corticosteroids and immunosuppressive agents as well as obliterative vessel involvement mark a difference to other connective tissue diseases. Frequent comorbidities such as osteoporosis, cancer, depression, atherosclerosis and malnutrition are additional factors contributing to a reduced quality of life. Systemic sclerosis therefore is a disease meeting the demands of an outpatient care according to § 116 b SGB V. In this article we discuss various aspects of disease management and care in specialized outpatient clinics.

Schlüsselwörter

Systemische Sklerose - Multiorganbeteiligung - spezialärztliche Versorgung

Keywords

Systemic sclerosis - multiorgan involvement - disease management
 

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