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

• Main Authors: Alessia Arduini (Author), Emiliano Marasco (Author), Giulia Marucci (Author), Manuela Pardeo (Author), Antonella Insalaco (Author), Ivan Caiello (Author), Gian Marco Moneta (Author), Giusi Prencipe (Author), Fabrizio De Benedetti (Author), Claudia Bracaglia (Author)
• Format: Book
• Published: BMC, 2019-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...