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

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 with diffe...

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Bibliographic Details
Published in:Pediatr Rheumatol Online J
Main Authors: Arduini, Alessia, Marasco, Emiliano, Marucci, Giulia, Pardeo, Manuela, Insalaco, Antonella, Caiello, Ivan, Moneta, Gian Marco, Prencipe, Giusi, De Benedetti, Fabrizio, Bracaglia, Claudia
Format: Artigo
Language:Inglês
Published: BioMed Central 2019
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Short Report
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC6532153/
https://ncbi.nlm.nih.gov/pubmed/31118063
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s12969-019-0328-3
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