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pp 1–8 | Cite as

Systemische Sklerose

Teil 1: Diagnose und Organmanifestationen
  • H. FindeisenEmail author
  • H. Grasshoff
  • G. Riemekasten
Kollagenosen
  • 4 Downloads

Zusammenfassung

Die systemische Sklerose ist eine seltene Autoimmunerkrankung – den Kollagenosen zugehörig. Vaskulopathie, Fibrose der Haut und Beteiligung der inneren Organe sind ausgesprochen heterogen und die Genese jener Krankheitsbilder bisher unverstanden. In den letzten Jahrzehnten sind einige Fortschritte im Management und in der Therapie erzielt worden, wobei weiterhin eher die Stabilisierung und Vermeidung von Krankheitssymptomen als die Verbesserung und Remissionserzielung erzielt werden können. In dieser Übersichtsarbeit möchten wir einen aktuellen Blick auf die wichtigen klinischen und therapeutischen Optionen werfen.

Schlüsselwörter

Systemische Sklerose Pulmonale arterielle Hypertonie Lungenfibrose Raynaud-Phänomen Sklerodermie 

Systemic sclerosis

Part 1: Diagnosis and Organ Manifestations

Abstract

Systemic sclerosis is a rare autoimmune disease which belongs to the group of connective tissue diseases. Vasculopathy, skin fibrosis and fibrosis of internal organs are heterogeneously expressed and the genesis of the disease is not fully understood. In the last decades some progress has been made in the disease management and treatment options, whereas the therapeutic aim is still stabilization of the disease and symptom control rather than disease improvement or remission. This review article presents a current perspective on the important clinical and therapeutic options.

Keywords

Systemic sclerosis Pulmonary arterial hypertension Lung fibrosis Raynaud phenomenon Scleroderma 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

H. Findeisen, H. Grasshoff und G. Riemekasten geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Copyright information

© Springer-Verlag GmbH Austria, ein Teil von Springer Nature 2020

Authors and Affiliations

  1. 1.Klinik für Rheumatologie und ImmunologieUniversitätsklinikum Schleswig-Holstein LübeckLübeckDeutschland

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