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Arthritis und Rheuma 2025; 45(03): 178-190
DOI: 10.1055/a-2489-7269
Schwerpunkt

Leitsymptome und Leitbefunde der systemischen Sklerose

Pascal van Wijnen
Justus-Liebig-Universität Gießen, Campus Kerckhoff, Abteilung für Rheumatologie, klinische Immunologie, Osteologie und Physikalische Medizin, Kerckhoff Klinik, Bad Nauheim
,
Philipp Klemm 
Justus-Liebig-Universität Gießen, Campus Kerckhoff, Abteilung für Rheumatologie, klinische Immunologie, Osteologie und Physikalische Medizin, Kerckhoff Klinik, Bad Nauheim
,
Ulf Müller-Ladner
Justus-Liebig-Universität Gießen, Campus Kerckhoff, Abteilung für Rheumatologie, klinische Immunologie, Osteologie und Physikalische Medizin, Kerckhoff Klinik, Bad Nauheim
,
Ingo Tarner
Justus-Liebig-Universität Gießen, Campus Kerckhoff, Abteilung für Rheumatologie, klinische Immunologie, Osteologie und Physikalische Medizin, Kerckhoff Klinik, Bad Nauheim
,
Nils Schulz
Justus-Liebig-Universität Gießen, Campus Kerckhoff, Abteilung für Rheumatologie, klinische Immunologie, Osteologie und Physikalische Medizin, Kerckhoff Klinik, Bad Nauheim
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Zusammenfassung

Die systemische Sklerose (SSc) ist eine seltene autoimmune Systemerkrankung mit komplexer Pathogenese, die durch das Zusammenspiel von Vaskulopathie, Inflammation, Autoimmunität und Fibrose geprägt ist. Trotz großer Fortschritte der Diagnostik und Therapie kann die SSc eine erhebliche Krankheitslast sowie eine erhöhte Mortalität der Betroffenen verursachen. Zu den Leitsymptomen zählen vor allem die Dermatosklerose und das Raynaud-Syndrom. Häufig lässt sich die Verdachtsdiagnose bereits durch eine ausführliche Anamnese und eine sorgfältige körperliche Untersuchung stellen. Wegweisende Befunde wie Hautveränderungen, Mikrostomie, Zungenbandsklerose und vaskuläre Manifestationen (z. B. digitale Ulzera) spielen dabei eine zentrale Rolle. Angesichts der potenziellen Krankheitsprogression und der Vielzahl möglicher Organmanifestationen (mukokutan, kardiopulmonal, gastrointestinal, muskuloskelettal, renal) sind ein frühzeitiges Screening und eine regelmäßige Überwachung unerlässlich. Ein interdisziplinärer Therapieansatz, der auf die Kontrolle von Inflammation, die Hemmung der Fibrose und die Verbesserung der Perfusion abzielt, ist von entscheidender Bedeutung. Ziel ist es, Organbeteiligungen zu minimieren, die Lebensqualität der Patienten zu steigern und die Sterblichkeit zu senken.

Schlüsselwörter

Dermatosklerose - interstitielle Lungenerkrankung - Kollagenose - Raynaud-Syndrom - systemische Sklerose


Publication History

Article published online:
02 June 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

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