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Paediatric sarcoidosis

Calender, Alain; Cavalin, Catherine; Clement, Annick; Macchi, Odile; Nathan, Nadia; Pacheco, Yves; Rosental, Paul-André; Sileo, Chiara; Valeyre, Dominique (2019), Paediatric sarcoidosis, Paediatric Respiratory Reviews, 29, p. 53-59. 10.1016/j.prrv.2018.05.003

Type
Article accepté pour publication ou publié
Date
2019
Journal name
Paediatric Respiratory Reviews
Volume
29
Pages
53-59
Publication identifier
Metadata
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Author(s)
Calender, Alain
Service de Génétique Moléculaire et Clinique
Cavalin, Catherine
Institut de Recherche Interdisciplinaire en Sciences Sociales [IRISSO]
Clement, Annick

Macchi, Odile
Centre d'études européennes et de politique comparée (Sciences Po, CNRS) [CEE]
Nathan, Nadia

Pacheco, Yves
Immunovirologie et polymorphisme génétique
Rosental, Paul-André
Centre d'études européennes et de politique comparée (Sciences Po, CNRS) [CEE]
Sileo, Chiara

Valeyre, Dominique
Service des maladies respiratoires
Abstract (EN)
Paediatric sarcoidosis is an extremely rare disease characterized by a granulomatous inflammation. The estimated incidence is 0.6–1.02/100,000 children, but in the absence of international registers, the disease is probably under-reported. Its pathophysiologic basis is not clearly understood but the current hypothesis is a combination of a genetic predisposition and an environmental exposure that could be either organic or mineral. Contrary to adult forms of the disease, general symptoms are often at the forefront at diagnosis. In its most frequent form, paediatric sarcoidosis is a multi-organ disorder affecting preferentially the lungs, the lymphatic system and the liver, but all organs can be affected. This review aims to provide an overview of current knowledge on sarcoidosis in children, providing a summary of the data available from cohort studies on the presentation, the management and the evolution of the disease in this specific population.
Subjects / Keywords
disease