An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome

An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome

Abstract Background Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition that presents with fever, rash and arthritis. At onset systemic features are predominant and the diagnosis may be a challenge. Secondary hemophagocytic lymphohistiocytosis (sHLH) forms may be associated wi...

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Bibliographic Details
Main Authors: Alessia Arduini, Emiliano Marasco, Giulia Marucci, Manuela Pardeo, Antonella Insalaco, Ivan Caiello, Gian Marco Moneta, Giusi Prencipe, Fabrizio De Benedetti, Claudia Bracaglia
Format: Article
Language:English
Published: BMC 2019-05-01
Series:Pediatric Rheumatology Online Journal
Subjects:
Macrophage activation syndrome
Juvenile idiopathic arthritis
Interferons
Interleukins
Chemokines
Online Access:http://link.springer.com/article/10.1186/s12969-019-0328-3

Internet

http://link.springer.com/article/10.1186/s12969-019-0328-3

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