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Review
. 2024 Mar;75(3):181-196.
doi: 10.1007/s00105-024-05298-8. Epub 2024 Feb 16.

[Systemic sclerosis]

[Article in German]
A-M Faber  1 , T Krieg  2 , N Hunzelmann  3
Affiliations
Review

[Systemic sclerosis]

[Article in German]
A-M Faber et al. Dermatologie (Heidelb). 2024 Mar.

Abstract
in English, German

Background: Systemic sclerosis (SSc) is a very heterogeneous, chronic, rare, but socioeconomically important disease with a severe disease course and severe impairment of the quality of life of affected patients.

Objectives: Overview of the current state of research on the pathogenesis, diagnosis and therapy of SSc.

Methods: A literature search was performed.

Results: The pathogenesis of SSc is not fully understood. ACR/EULAR criteria allow the diagnosis of early forms of SSc. Classification into limited cutaneous SSc and diffuse cutaneous SSc is of prognostic and therapeutic relevance. New organ-specific treatment options for SSc have led to improved quality of life and prognosis.

Zusammenfassung: HINTERGRUND: Die systemische Sklerose (SSc) stellt eine sehr heterogene, chronische, zwar seltene, jedoch sozioökonomisch bedeutsame Erkrankung mit schwerem Krankheitsverlauf und starker Einschränkung der Lebensqualität betroffener Patienten dar.

Fragestellung: Es wird eine Übersicht zum aktuellen Forschungsstand zu Pathogenese, Diagnostik und Therapie der SSc gegeben.

Material und methode: Es erfolgte eine Literaturrecherche.

Ergebnisse: Die Pathogenese der SSc bleibt bislang weiterhin nicht gänzlich geklärt. ACR(American College of Rheumatology)/EULAR(European League Against Rheumatism)-Kriterien erlauben eine Diagnose auch früher SSc-Formen. Die Klassifikation in limitierte kutane systemische Sklerose (lcSSc) und diffuse kutane systemische Sklerose (dcSSc) ist von prognostischer und therapeutischer Relevanz. Neue organspezifische Therapiemöglichkeiten der SSc haben zu einer verbesserten Lebensqualität und Prognose geführt.

Keywords: Connective tissue hyperplasia; Interdisciplinary health team; Organ-specific treatment; Quality of life; Scleroderma.

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References

Literatur

    1. Bergamasco A et al (2019) Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol 11:257–273 - PubMed - PMC
    1. Jerjen R et al (2022) Systemic sclerosis in adults. Part I: clinical features and pathogenesis. J Am Acad Dermatol 87(5):937–954 - PubMed
    1. Arnett FC et al (2001) Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis Rheum 44(6):1359–1362 - PubMed
    1. Rosendahl AH, Schonborn K, Krieg T (2022) Pathophysiology of systemic sclerosis (scleroderma). Kaohsiung J Med Sci 38(3):187–195 - PubMed
    1. De Martinis M et al (2016) An overview of environmental risk factors in systemic sclerosis. Expert Rev Clin Immunol 12(4):465–478 - PubMed

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