The puzzling clinical spectrum and course of juvenile sarcoidosis
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The puzzling clinical spectrum and course of juvenile sarcoidosis Andrew Fretzayas, Maria Moustaki, Olga Vougiouka Athens, Greece
Data sources: PubMed database was searched using terms sarcoidosis, children or childhood sarcoidosis or juvenile sarcoidosis in combination with one of the following terms: epidemiology, clinical manifestations, genetics, diagnosis, treatment, and prognosis. We also retrieved the terms such as early onset sarcoidosis and Blau syndrome. Furthermore, e-medicine and European Respiratory Society monographs for sarcoidosis were reviewed. Results: Sarcoidosis in childhood presents with two age dependent, distinct forms. In younger children it is clinically evident before the age of four years and characterized by the triad of rash, arthritis and uveitis. In their older counterparts, the juvenile late onset sarcoidosis involves several organs and its clinical appearance resembles the adult type of the disease, with the respiratory system being most frequently affected (hilar lymphadenopathy, pulmonary infiltrations). Steroid is the main agent of treatment whereas methotrexate is also used for beneficial steroid sparing effects. New, novel therapies may change the outcome of the disease especially in difficult morbid cases. Conclusions: Sarcoidosis in childhood is recognized as a systemic disease affecting various organs and having diverse clinical course depending on the age of onset. World J Pediatr 2011;7(2):103-110
Author Affiliations: Third Department of Pediatrics, University of Athens, School of Medicine "Attikon" University Hospital, Athens, Greece (Fretzayas A, Moustaki M); Second Department of Pediatrics, University of Athens, School of Medicine, "P & A Kyriakou" Children's Hospital, Athens, Greece (Vougiouka O) Corresponding Author: Andrew Fretzayas, MD, PhD, Third Department of Pediatrics, "Attikon" University Hospital, Athens University School of Medicine,1 Rimini str, Haidari, 12462 Athens, Greece (Tel: 00302105831299; Fax: 00302105832229; Email: [email protected]) doi: 10.1007/s12519-011-0261-0 ©Children's Hospital, Zhejiang University School of Medicine, China and Springer-Verlag Berlin Heidelberg 2011. All rights reserved.
World J Pediatr, Vol 7 No 2 . May 15, 2011 . www.wjpch.com
Key words: corticosteroids; early onset sarcoidosis; juvenile sarcoidosis; late onset sarcoidosis; methotrexate
Introduction
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ince Hutchinson's first description of the skin manifestations of sarcoidosis in 1885 and subsequent reports of the histopathologic pattern of the lesion and appearance of the term "sarkoid" in 1899, several investigators have greatly contributed to the current understanding of the disease although many aspects have not been elucidated.[1] Juvenile sarcoidosis (JS) is a chronic, multisystem, granulomatous disease of obscure etiology occurring rarely throughout childhood.[2-4] Clinical manifestations vary according to the organs involved. Two age dependent, distinct types of the disease have been recognized in children.[3,4] In the youngsters the clinical triad of r
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