Juvenile systemische Sklerodermie

ZUSAMMENFASSUNG

Die juvenile systemische Sklerodermie (jSSc) ist eine seltene Erkrankung mit teilweise schwerer Organbeteiligung. Eine frühe Erkennung der Organbeteiligungen ist wichtig, um Schäden zu verhindern. Die juvenile systemische Sklerodermie Inzeptionskohorte ist eine Kohorte von jSSc-Patienten und derzeit die größte Kohorte der Welt. In dieser Publikation werden die Charakteristika von 210 Patienten dargestellt, die aus der Datenbank bis 12/2021 extrahiert wurden. Es wird die aktuelle Erfassung der Organbeteiligung vorgestellt. jSSc-Patienten unterscheiden sich von Patienten, die im Erwachsenenalter erkranken. Die jSSc-Patienten mit diffusem Subset haben einen schwereren Verlauf.

ABSTRACT

Juvenile systemic scleroderma is an orphan disease, with partially severe organ involvement. Early diagnosis of the organ involvements is essential to prevent damage. The juvenile systemic scleroderma inception cohort is a cohort of juvenile systemic scleroderma patients worldwide. It is currently the largest cohort of the world. In this paper the patients characteristics of 210 patients, data extracted till 12/2021 and assessment of the patients is presented. Juvenile systemic scleroderma patients differ from the adult onset patients and diffuse subset patients have a more severe course.

Schlüsselwörter

Juvenile systemische Sklerodermie - Organbeteiligung - Diagnostik

Keywords

Juvenile systemic scleroderma - organ involvements - diagnostics

Publication History

Article published online:
05 November 2022

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