TITLE:
Juvenile Systemic Sclerosis: About 9 Cases
AUTHORS:
Kaoutar Danaoui, Houda Nassih, Khadija Oujennane, Rabiy El Qadiry, Aicha Bourrahouat, Said Amal, Imane Ait Sab
KEYWORDS:
Scleroderma, Systemic Sclerosis, Children, Pediatric
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.14 No.2,
March
14,
2024
ABSTRACT: Scleroderma is a rare disease with two primary
forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are
chronic conditions that can manifest in
various patterns (subtypes) and are linked to extracutaneous involvement
in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening
complications such as lung, heart, and visceral organ fibrosis, and
vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but
patients are susceptible to significant morbidity, leading to severe
disfigurement and functional impairment. Treatment for scleroderma aims to
control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts
a retrospective descriptive analysis aiming to document the clinical
manifestations, management approaches, and outcomes of systemic sclerosis in a
cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University,
Hospital Center in Marrakech, Morocco.